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(1) Background: Mastery of auscultation can be challenging for many healthcare providers. Artificial intelligence (AI)-powered digital support is emerging as an aid to assist with the interpretation of auscultated sounds. A few AI-augmented digital stethoscopes exist but none are dedicated to pediatrics. Our goal was to develop a digital auscultation platform for pediatric medicine. (2) Methods: We developed StethAid—a digital platform for artificial intelligence-assisted auscultation and telehealth in pediatrics—that consists of a wireless digital stethoscope, mobile applications, customized patient-provider portals, and deep learning algorithms. To validate the StethAid platform, we characterized our stethoscope and used the platform in two clinical applications: (1) Still’s murmur identification and (2) wheeze detection. The platform has been deployed in four children’s medical centers to build the first and largest pediatric cardiopulmonary datasets, to our knowledge. We have trained and tested deep-learning models using these datasets. (3) Results: The frequency response of the StethAid stethoscope was comparable to those of the commercially available Eko Core, Thinklabs One, and Littman 3200 stethoscopes. The labels provided by our expert physician offline were in concordance with the labels of providers at the bedside using their acoustic stethoscopes for 79.3% of lungs cases and 98.3% of heart cases. Our deep learning algorithms achieved high sensitivity and specificity for both Still’s murmur identification (sensitivity of 91.9% and specificity of 92.6%) and wheeze detection (sensitivity of 83.7% and specificity of 84.4%). (4) Conclusions: Our team has created a technically and clinically validated pediatric digital AI-enabled auscultation platform. Use of our platform could improve efficacy and efficiency of clinical care for pediatric patients, reduce parental anxiety, and result in cost savings.

Double aortic arch associated with atresia of the left arch proximal to the left common carotid artery has been considered a theoretical possibility. To our knowledge, we report the first patient with this anatomy confirmed by surgical observation.

To assess the role of neonatal pulse oximetry screening and other bases of referral for patients of all ages with aortic coarctation referred to a tertiary care medical center.Medical records were reviewed for 200 consecutive patients diagnosed and treated for CoA in either 2006-2011 or 2015-2019, before and after mandated pulse oximetry screening, respectively.In both groups, ∼50% of patients were diagnosed within the first 5 days. Diagnosis by fetal echocardiography was more frequent in the 2015-2019 group (30.5% vs 20.5%; P .03); obstruction often developed only as the ductus arteriosus closed. In each group, ∼25% of patients were diagnosed at age1 year and 7% had an incidental diagnosis. Pulse oximetry screening was documented as abnormal in only 8 of 47 patients and was not performed in those with a fetal diagnosis. Evaluation of a murmur was the second most frequent basis for referral. Moderate to severe left ventricular dysfunction occurred mainly in infants in the first month, with a similar frequency in the 2 groups; these patients often had tachypnea or poor weight gain. Decreased femoral pulses or systemic hypertension were infrequently documented by referring physicians. Hypertension typically was ascribed to a renal or essential basis. Exercise symptoms occurred mainly in patients age10 years.Although fetal echocardiography and neonatal pulse oximetry contribute to the diagnosis of coarctation, physical examination has an important complementary role. Evaluation of peripheral pulses on initial and early follow-up neonatal examinations, along with consideration of coarctation in any patient with hypertension, are needed to improve timely detection.

Objective A review of our center's experience before March 2011 showed that one half of 36 patients who had a baffling or reimplantation procedure to repair scimitar syndrome developed pulmonary vein obstruction. We analyzed the results of a new operation that enlarges the left atrium and avoids circuitous pathways or tension on the scimitar pulmonary vein. Methods Between April 2011 and November 2018, 22 patients underwent scimitar vein surgery; 11 had baffling or reimplantation and 11 only had the new operation that included resection of the atrial septum with removal of the muscular limbus. The left atrium was pulled down toward the scimitar vein and a V-shaped incision made at the scimitar vein atrial junction with the space filled with a pulmonary homograft. If the scimitar vein coursed adjacent to the atrium, a V-shaped incision was made into the scimitar vein and directly anastomosed to the atrium. A patch of autologous pericardium was used to septate the atrium and an additional patch placed anteriorly to augment the inferior vena cava. Results Of the 11 patients who had baffling or reimplantation, 5 developed pulmonary vein obstruction between 45 days and 9.5 months after surgery associated with baffle thrombosis or tension on the pulmonary vein. None of the 11 patients who only had the new procedure developed pulmonary vein obstruction during postoperative monitoring up to 3.6 years. Conclusions Patients having only the multipatch procedure for repair of scimitar syndrome have not developed postoperative pulmonary vein obstruction in the short to intermediate term.

Objectives To determine if patients evaluated using the pediatric chest pain standardized clinical assessment and management plan (SCAMP) in cardiology clinic were later diagnosed with unrecognized cardiac pathology, and to determine if other patients with cardiac pathology not enrolled in the SCAMP would have been identified using the algorithm. Study Design Patients 7–21 years of age, newly diagnosed with hypertrophic or dilated cardiomyopathy, coronary anomalies, pulmonary embolus, pulmonary hypertension, pericarditis, or myocarditis were identified from the Boston Children's Hospital (BCH) cardiac database between July 1, 2010 and December 31, 2012. Patients were cross-referenced to the SCAMP database or retrospectively assessed with the SCAMP algorithm. Results Among 98 patients with cardiac pathology, 34 (35%) reported chest pain, of whom 10 were diagnosed as outpatients. None of these patients were enrolled in the SCAMP because of alternate chief complaints (n = 4) or referral to BCH for management of the new diagnosis (n = 6). Each of these patients would have had an echocardiogram recommended by retrospective application of the SCAMP algorithm. Two other patients with cardiac pathology were among the 1124 patients assessed by the SCAMP. One patient initially diagnosed with noncardiac chest pain presented 18 months later and was diagnosed with myocarditis as an inpatient. One patient seen initially in the emergency department was subsequently diagnosed with pericarditis as an outpatient. Conclusions Patients assessed by the chest pain SCAMP at BCH were not later diagnosed with cardiac pathology that was missed at the initial encounter. Nonenrolled outpatients with cardiac pathology and chest pain would have been successfully identified with the SCAMP algorithm.

The diagnosis of secundum atrial septal defect often is delayed. For 310 patients with hemodynamically significant secundum atrial septal defect undergoing closure over a 5-year period at a single medical center, this study reviews the symptoms prompting referral, limitations of physical examination and electrocardiography, and basis for initially missing the diagnosis.

BACKGROUND: Delayed diagnosis of critical congenital heart disease (CCHD) in neonates increases morbidity and mortality. The use of pulse oximetry screening is recommended to increase detection of these conditions. The contribution of pulse oximetry in a tertiary-care birthing center may be different from at other sites. METHODS: We analyzed CCHD pulse oximetry screening for newborns ≥35 weeks’ gestation born at Brigham and Women’s Hospital and cared for in the well-infant nursery during 2013. We identified patients with prenatal diagnosis of CCHD. We also identified infants born at other medical centers who were transferred to Boston Children’s Hospital for CCHD and determined if the condition was diagnosed prenatally. RESULTS: Of 6838 infants with complete pulse oximetry data, 6803 (99.5%) passed the first screening. One infant failed all 3 screenings and had the only echocardiogram prompted by screening that showed persistent pulmonary hypertension. There was 1 false-negative screening in an infant diagnosed with interrupted aortic arch. Of 112 infants born at Brigham and Women’s Hospital with CCHD, 111 had a prenatal diagnosis, and none was initially diagnosed by pulse oximetry. Of 81 infants transferred to Boston Children’s Hospital from other medical centers with CCHD, 35% were diagnosed prenatally. CONCLUSIONS: In our tertiary-care setting, pulse oximetry did not detect an infant with CCHD because of effective prenatal echocardiography screening. Pulse oximetry will detect more infants in settings with a lower prenatal diagnosis rate. Improving training in complete fetal echocardiography scans should also improve timely diagnosis of CCHD.

BACKGROUND AND OBJECTIVES:Chest pain is a complaint for which children are frequently evaluated. Cardiac causes are rarely found despite expenditure of considerable time and resources. We describe validation throughout New England of a clinical guideline for cost-effective evaluation of pediatric patients first seen by a cardiologist for chest pain using a unique methodology termed the Standardized Clinical Assessment and Management Plans (SCAMPs).METHODS:A total of 1016 ambulatory patients, ages 7 to 21 years initially seen for chest pain at Boston Children’s Hospital (BCH) or the New England Congenital Cardiology Association (NECCA) practices, were evaluated by using a SCAMPs chest pain guideline. Findings were analyzed for diagnostic elements, patterns of care, and compliance with the guideline. Results from the NECCA practices were compared with those of Boston Children’s Hospital, a regional core academic center.RESULTS:Two patients had chest pain due to a cardiac etiology, 1 with pericarditis and 1 with an anomalous coronary artery origin. Testing performed outside of guideline recommendations demonstrated only incidental findings. Patients returning for persistent symptoms did not have cardiac disease. The pattern of care for the NECCA practices and BCH differed minimally.CONCLUSIONS:By using SCAMPs methodology, we have demonstrated that chest pain in children is rarely caused by heart disease and can be evaluated in the ambulatory setting efficiently and effectively using minimal resources. The methodology can be implemented regionally across a wide range of clinical practice settings and its approach can overcome a number of barriers that often limit clinical practice guideline implementation.

Scimitar syndrome is a rare congenital anomaly. We evaluated risk factors for postoperative pulmonary vein stenosis or death and predictive factors for survival without scimitar vein surgery in patients with scimitar syndrome.The records of patients with scimitar syndrome evaluated at our medical center between 1964 and 2011 were reviewed.Scimitar syndrome was identified in 80 patients, with a median follow-up of 4.5 years. Patients presenting less than 1 year of age had a higher incidence of symptoms, aortopulmonary collaterals, coexisting congenital heart disease (CHD), extracardiac anomalies, and pulmonary hypertension. Of 36 patients having scimitar vein surgery, 18 had postoperative pulmonary vein obstruction that occurred with similar frequency after baffle or reimplantation procedures, early or late in the study period, and tended to be more common in infants (P = .10). Overall, 19 (24%) of 80 died. Multivariate risk factors for death included systolic pulmonary pressure0.5 systemic level (P = .007) and left pulmonary vein stenosis (P = .009). Pulmonary artery systolic pressure0.5 systemic level (P = .01) and absence of CHD excluding atrial septal defect (P = .01) were predictive factors in 28 patients who survived and did not have scimitar vein surgery; these patients had no or mild right ventricular dilation and a ratio of pulmonary-to-systemic flow1.6 either at baseline, after coiling aortopulmonary collaterals or nonscimitar vein intervention.Postoperative pulmonary vein obstruction is common after scimitar vein surgery regardless of redirection technique. Pulmonary hypertension and left pulmonary vein stenosis are risk factors for death, whereas patients without significant pulmonary hypertension or associated CHD did well without scimitar vein surgery. These observations may guide management decisions in patients with scimitar syndrome.

Objective: To review the short and medium term outcome of transcatheter pulmonary valve perforation (PVP) in patients with pulmonary atresia-intact ventricular septum and non-right ventricular dependant coronary circulation (PA/IVS non-RVDCC).Background: PVP in patients with PA/IVS non-RVDCC has become more common in the past two decades. However, data on outcomes with this strategy are mixed.Methods: Data were reviewed retrospectively for all patients with PA/IVS non-RVDCC treated from 1996- 2010 at our institution. Patients who had severe neonatal Ebstein malformation, or initial interventional management at another institution were excluded.Results: PVP was attempted in 30 of 50 patients (60%); 26 (87%) of these had a successful procedure. Twenty-four patients (48%) had surgery without PVP. There were no deaths in the cohort. Complications of PVP included 5 (17%) myocardial perforations. Of those with successful PVP, 10 (38%) did not have surgery (PVP-NS) and 16 (62%) had surgery (PVP-S) prior to discharge. Tricuspid valve (TV)Z-score was larger in the PVP-NS than in PVP-S patients, with median TV diameterZ-scores of10.7 (20.9, 1.7) and21.1 (-2.8, 2), respectively (P50.01). Time from PVP to either hospital discharge (PVP-NS group) or surgery (PVP-S group) was significantly different between groups: 15 (7, 22) and 8 days (0, 46), respectively (P50.01). There were no differences in the number of trials or lowest arterial PaO2 off prostaglandins between groups. All patients in the PVP-NS group had a biventricular circulation at a median follow-up of 4.3 years. Conclusions: The results of a collaborative approach to treating neonates with PA/IVS non-RVDCC are excellent. Smaller TV size is associated with greater likelihood of surgery prior to discharge, and may serve as a surrogate for early RV inadequacy. V C 2012 Wiley Periodicals Inc.

Introduction. Chest pain in children is common, but rarely heralds serious underlying cardiac pathology. Despite this, the anxiety of missing a potentially life threatening condition creates a large burden of referrals and diagnostic testing. We evaluated patients diagnosed with 1 of 9 serious cardiac diseases and detailed the clinical signs and symptoms of the patients presenting with chest pain. Methods. Patients diagnosed between the ages of 7 and 21 years from January 2000 to December 2009 at Children’s Hospital Boston (CHB) were identified from a database using diagnostic and billing codes for aortic dissection, coronary anomalies, dilated cardiomyopathy, hypertrophic cardiomyopathy, myocarditis, pericarditis, pulmonary embolus, pulmonary hypertension, and Takayasu arteritis. Patients with previously diagnosed congenital or acquired heart disease were excluded. Results. Four hundred eighty-four patients were included and 35% presented with chest pain. Forty-one (24%) of these patients with chest pain were diagnosed in the outpatient cardiology clinic, while the remaining 130 patients (76%) were diagnosed in the emergency department (ED) or inpatient setting. Coronary artery anomalies were the most common diagnosis made in cardiology clinic, and 16 of the 23 (70%) patients with serious coronary anomalies had exercise-induced chest pain. Patients presenting to the ED or inpatient units tended to have other important nonspecific symptoms (35–44%), high-risk past medical histories (12%), physical examination findings (32%), and electrocardiogram (ECG) abnormalities (78%) that heighten clinical suspicion of cardiac disease. Conclusions. Identifying underlying cardiac pathology in the CHB outpatient cardiology department in patients presenting with chest pain is rare, with only 41 cases over a 10-year period. The presence of exertional chest pain was important in identifying patients with coronary artery anomalies. A detailed history and physical examination, along with a critical review of an ECG, seem to identify those patients with rare diseases who need further diagnostic testing.

Clinical practice is discrepant regarding routine enlargement of the interatrial communication (IAC) in patients with right-sided atrioventricular valve atresia. We determined the percentage and risk factors of those who develop a restrictive IAC.Medical records were reviewed for patients treated from 1985 to 2006, including those admitted in the first 6 weeks of life (group A), and those referred at a later age (group B), some of whom had routine atrial septal procedures. In group A, we analyzed the initial postnatal echocardiogram.Group A consisted of 79 patients, 16 of whom had an atrial septectomy as part of the initial surgical procedure. Of the remaining 63 patients, 9 (14%) developed atrial septal restriction. In group A, an atrial septal aneurysm (ASA) (OR 16, P = .006) and IAC diameter5 mm (OR 13, P = .009) were associated with atrial septal restriction. Atrial septal restriction occurred in 80% of patients with both features, 20% with IACor = 5 mm and ASA, 18% with IAC5 mm and no ASA, and 2% with neither feature. Group B consisted of 95 patients, 27 of whom had an atrial septal procedure. Of the remaining 68 patients, 11 (16%) developed atrial septal restriction.Routine enlargement of the IAC is not necessary in patients with right-sided atrioventricular valve atresia. Patients with ASA or IAC5 mm are at increased risk for development of atrial septal restriction.

Objectives The purpose of this research was to investigate the causes and symptoms of superior vena cava (SVC) obstruction or occlusion and report on the long-term results of transcatheter therapy. Background Information on transcatheter therapy for SVC obstruction is limited. Methods Superior vena cava catheterization interventions between August 1984 and April 2006 were reviewed. Patients were divided into 2 subgroups depending on whether or not they had previously undergone congenital cardiac surgery. Results Sixty-three patients with median age of 3.7 years (range 1 month to 42 years) and weight of 13.3 kg (range 3 to 114 kg) were treated. Fifty patients (79%) were symptomatic, although only 50% had symptoms suggestive of SVC obstruction. Superior vena cava syndrome was more common in the non-cardiac surgical group (52% vs. 10%, p = 0.001). The mean gradient and SVC diameter improved from 10.8 ± 5.8 mm Hg to 2.6 ± 2.2 mm Hg (p 5 years at the time of intervention. Conclusions Superior vena cava-related symptoms occur in only 50% of patients with hemodynamically significant SVC obstruction. Endovascular therapy is successful in relieving the stenosis and associated symptoms with good long-term results.

To determine if patients evaluated using the pediatric chest pain standardized clinical assessment and management plan (SCAMP) in cardiology clinic were later diagnosed with unrecognized cardiac pathology, and to determine if other patients with cardiac pathology not enrolled in the SCAMP would have been identified using the algorithm.Patients 7-21 years of age, newly diagnosed with hypertrophic or dilated cardiomyopathy, coronary anomalies, pulmonary embolus, pulmonary hypertension, pericarditis, or myocarditis were identified from the Boston Children's Hospital (BCH) cardiac database between July 1, 2010 and December 31, 2012. Patients were cross-referenced to the SCAMP database or retrospectively assessed with the SCAMP algorithm.Among 98 patients with cardiac pathology, 34 (35%) reported chest pain, of whom 10 were diagnosed as outpatients. None of these patients were enrolled in the SCAMP because of alternate chief complaints (n = 4) or referral to BCH for management of the new diagnosis (n = 6). Each of these patients would have had an echocardiogram recommended by retrospective application of the SCAMP algorithm. Two other patients with cardiac pathology were among the 1124 patients assessed by the SCAMP. One patient initially diagnosed with noncardiac chest pain presented 18 months later and was diagnosed with myocarditis as an inpatient. One patient seen initially in the emergency department was subsequently diagnosed with pericarditis as an outpatient.Patients assessed by the chest pain SCAMP at BCH were not later diagnosed with cardiac pathology that was missed at the initial encounter. Nonenrolled outpatients with cardiac pathology and chest pain would have been successfully identified with the SCAMP algorithm.

A persistent left superior vena cava connection to an unroofed coronary sinus is a rare cardiac anomaly that is associated with a variable degree of cyanosis. We report an infant with this condition and the unusual feature of cyanosis dependent on head position. When the patient's head was rotated to the left, he developed severe stenosis of the left internal jugular vein, enlarged cervical collateral veins that connected to the right superior vena cava and had an oxygen saturation 95%. When the patient's head was rotated to the right, the left internal jugular vein was widely patent and systemic oxygen saturation decreased to 87%. There was no right ventricular volume overload. Temporary occlusion of the left superior vena cava documented tolerable proximal venous pressure. Cyanosis was relieved by transcatheter closure of the left superior vena cava with a Gianturco-Grifka vascular occlusion device. Cathet. Cardiovasc. Intervent. 48:369-373, 1999.

We report a 52-year-old adult with Ebstein's anomaly associated with severe valvar pulmonary stenosis, secundum atrial septal defect, polycythemia, hypoxemia, and severe exertional limitations. Balloon pulmonary valvuloplasty relieved the valvar obstruction, reduced the degree of tricuspid regurgitation and polycythemia, and improved systemic oxygenation and exercise abilities. Use of this technique in patients with this combination of lesions can postpone the need for surgical intervention.

Surgical angioplasty for branch pulmonary arterial stenosis (BPAS) is associated with a low success rate and a high mortality rate. The success rate of balloon angioplasty using low pressure balloon catheters is about 60%. Little data are available on the success rate using high-pressure balloon (HPB) catheters. Therefore, our goal is to report the procedural and mid-term success rates using HPB catheters for the treatment of BPAS and those factors associated with success. 38 patients (25 boys/13 girls) with BPAS underwent at least one attempt (48 procedures) of balloon angioplasty using HPB catheters. Their median age was 1.1 years (range, 0.1–22.1 year), and their median weight was 9.6 kg (range, 3.7–61 kg). Thirty patients (79%) had BPAS following surgical intervention for their cardiac defects (mostly post tetralogy of Fallot), and eight (21%) patients had the BPAS associated with Williams syndrome (2 patients), Alagille syndrome (one patient), unoperated tetralogy of Fallot (one patient), and congenital BPAS (4 patients). Twenty-six procedures involved the left pulmonary artery (LPA), and 22 procedures involved the right pulmonary artery (RPA). The overall acute success rate was 56% (27 of 48 vessels). The mean ± SD of the gradient across the stenotic segment decreased from 37 ± 20 to 23 ± 20 mmHg (P < 0.001), and the diameter of the narrowest segment improved from 3.5 ± 1.6 to 5.7 ± 2.5 mm (P < 0.001). The median RV/AO pressure ratio decreased from 0.64 to 0.52 (P = 0.009). The median ratio of balloon size to the diameter of the narrowest segment was 2.67 (range, 1.3–7.8). Lesion location (RPA) was associated with higher acute success rate (P = 0.03), but not with mid-term outcome. Acute (P = 0.006) and mid-term (P = 0.04) success were related to balloon diameter to vessel diameter ratio. At mid-term follow-up (median interval 2.5 years), 16 vessels (33%) were widely patent. Complications included perforation of a small branch pulmonary artery in one patient, resulting in hemoptysis with complete recovery. One patient developed pulmonary artery hypertension; two patients developed an aneurysm of the dilated segment; one patient developed femoral vein thrombosis; and one patient had a paradoxical embolus associated with a cerbrovascular accident. There were no procedure-related deaths. We conclude that BPAS is challenging to treat. The use of HPB angioplasty may avoid surgery or the permanence of stem implantation and should be the first intervention for this condition in the pediatric patient. Other interventions should be reserved for refractory stenosis.

Late Sudden Death Risk in Postoperative TOF. Following surgery for tetralogy of Fallot (TOP), children may develop late onset ventricular arrhythmias. Many patients have both depolarization and repolarization abnormalities, including right bundle branch block (RBBB) and QT prolongation. The goal of this study was to improve prospective risk-assessment screening for late onset sudden death. Resting ECG markers including QRS duration, QTc, JTc, and interlead QT and JT dispersion were statistically analyzed to identify those patients at risk for ventricular arrhythmias and sudden cardiac death. To determine predictive markers for future development of arrhythmia, we examined 101 resting ECGs in patients (age 12 ± 6 years) with postoperative TOF and RBBB, 14 of whom developed late ventricular tachycardia (VT) or sudden death. These ECGs were also compared with an additional control group of 1000 age- and gender-matched normal ECGs. The mean QRS (± SD) in the VT group was 0.18 ± 0.02 seconds versus 0.14 ± 0.02 seconds in the non-VT group (P < 0.01). QTc and JTc in the VT group was 0.53 ± 0.05 seconds and 0.33 ± 0.03 seconds compared with 0.50 ± 0.03 seconds and 0.32 ± 0.03 seconds in the non-VT group (P = NS). There was no increase in QT dispersion among TOF patients with VT or sudden death compared with control patients or TOF patients without VT, although JT dispersion was more common in the TOF groups. A prolonged QRS duration in postoperative TOF with RBBB is more predictive than QTc, JTc, or dispersion indexes for identifying vulnerability to ventricular arrhythmias in this population, while retaining high specificity. The combination of both QRS prolongation and increased JT dispersion had very good positive and negative predictive values. These results suggest that arrhythmogenesis in children following TOF surgery might involve depolarization in addition to repolarization abnormalities. Prospective identification of high-risk children may be accomplished using these ECG criteria.

Balloon atrial septostomy remains an important interventional procedure in the pediatric age group. The incidence of potential complications using the conventional balloon ranges from 0–11%. The purpose of this study was to evaluate a new low-profile end-hole septostomy balloon catheter with dual lumen, inserted via a 5F or 6F sheath. Seventeen neonates and infants with various forms of congenital heart disease requiring palliation underwent septostomy using the new catheter at a median age of 19 days (with a range of 1–593 days), and a median weight of 3.4 kg (a range of 2.5–8.4 kg). The aortic saturation increased from 72 ± 20% to 87 ± 7%, p < .001; and the gradient across the atrial septum decreased (a-wave gradient from 11.2 ± 10.3 to 2.1 ± 3.6 mm Hg; v-wave from 10.4 ± 7.7 to 1.2 ± 1.2 mm Hg; and mean gradient from 8.5 ± 6.9 to 0.9 ± 1.3 mm Hg, p < .002). The diameter of the defect increased from 2.7 ± 1.7 mm to 8 ± 2.3 mm, p < .001. There were no complications. We conclude that this new low-profile septostomy catheter is safe and effective in creating a large defect size between the atria. Because of the smaller inflation size of the balloon and smaller introducer sheath compared with the conventional catheter, this new septostomy catheter should be especially useful in small neonates. Cathet. Cardiovasc. Diagn. 40:187–190, 1997. © 1997 Wiley-Liss, Inc.

There have been multiple modifications for surgical palliation of functional single ventricle since the initial report by Fontan and Baudet in 1971. A modified Fontan procedure has been offered to patients with a variety of complex cyanotic heart disease at younger ages, and has resulted in decreased mortality. Reducing morbidity continues to be a challenge. These issues are multiple and include effusions, arrhythmia, ventricular function, exercise ability, progressive cyanosis from pulmonary arteriovenous malformations, systemic to hepatic venovenous malformations or atrial level shunting, thromboembolism, and protein-losing enteropathy. This article reviews clinical contributions published in the past year.

Experience with transcatheter closure (TCC) of patent ductus arteriosus (PDA) in small infants is limited. Our goals were to evaluate the immediate and short-term results of TCC of PDA in small infants (or = 8 kg) as attempted with single or multiple Gianturco coils and to compare results for PDAor = 2.5 mm versus PDA2.5 mm. Twenty-four infants underwent an attempt at TCC of PDA at a median age of 0.7 years (range 2 weeks to 1.5 years) and median weight of 6.5 kg (range 2.3 to 8 kg). Coils were placed by the transvenous route in 14 patients and by the transarterial route in 10 patients. The median PDA diameter was 2.7 mm (range 0.4 to 5.2 mm). Ten patients had PDA diameters2.5 mm and all had complete closure, 9 with a single coil and 1 with two coils. Fourteen patients had PDA diametersor = 2.5 mm; closure was complete in 10 patients, 9 of whom required multiple coils. There was a trend toward improved results with multiple coils delivered transvenously. There was immediate complete closure of the PDA with a diameter as large as 5.2 mm that persisted at a median follow-up period of 1.2 year. Closure was unsuccessful in 4 patients, each of whom required multiple coils. The median fluoroscopy time was 19 minutes. Complications included transient loss of femoral pulse in 2 infants, mild left pulmonary artery obstruction in 2, and nonretrievable coil migration to the right lung in one. We conclude that TCC is effective therapy for small infants with a PDA of diameteror = 5.2 mm by the single-or multiple-coil technique on an outpatient basis and that TCC can be performed in neonates as small as 2.3 kg.

Our objective was to assess the immediate and short-term results of stent implantation to relieve pulmonary artery stenosis (PAS). Thirty-seven patients underwent an attempt at stent implantatlon at a medlan age of 7.0 years (range, 0.G31.4 years) and a median weight of 20.5 kg (range, 7.4-85 kg). Twenty-two patients had previous tetralogy of Fallot repair. A total of 55 stents were Implanted successfully in 36 patlents. The peak systolic gradient across the stenotic segment decreased from a mean of 43 2 20.4 mmHg prestent to 13 f 13.9 mmHg (P a:: 0.001) poststent. The diameter of the narrowest segment Increased from a mean of 4.8 f 1.6 mm to 10.5 f 2.6 mm (P ,:I 0,001). The right ventrlcular-to-aortic mean systolic pressure ratio decreased from 0.74 2 0.2 to 0.52 ? 0.1 9 (P c: 0.001). Complications included balloon rupture prior to full stent expansion in 4 patients (in 2 patients the stent was positioned in the superior vena cava, and in 2 in the inferior vena cava), distal migration of a stent which was successfully retrieved at surgery 1 mo later in 1 patient, and tethering of the stent to the balloon requiring surgical removal in 1 patient. One patient died several hours after stent placement. Sixteen patients underwent repeat catheterization at a mean follow-up interval 010.9 * 0.5years (range, 0.2-2.0years). The mean gradient across the stent for these 16 patients was 26.7 f 19.8 mmHg, and there was no change in the mean diameter (9.4 2 3.2 mm). Two patients developed stenosis related to neointimal proliferation at the stent site which was redilated successfully. In conclusion, stent irnplantation is generally safe and effective in relleving PAS. c 1996 WIIO~-LI~~. Inc

We report two infants each with coarctation of the aorta and small patent ductus arteriosus who had balloon angioplasty therapy for aortic obstruction at 3-6 months of age followed by anterograde placement of a single Gianturco coil to close the ductus at a separate catheterization. One infant developed recoarctation that resolved with repeat angioplasty prior to closure of the ductus. Follow-up evaluations have demonstrated a trivial aortic gradient, no aortic aneurysm, and no residual shunt.

Transcatheter closure of a patent ductus arteriosus (PDA) using the Rashkind occluder or the buttoned device is accompanied with a 7–38% incidence of residual shunt. Closure of the residual shunt with a second device is expensive, associated with possible morbidity, can be technically challenging, and occasionally does not completely eliminate the shunt. Our objective was to assess the immediate and short-term results of transcatheter closure of residual PDA after the Rashkind device using single or multiple Gianturco coils. Five patients (one male/four female) underwent transcatheter closure of residual PDA at a median age of 8.4 yr (range 2–10.1 yr) and median weight of 23 kg (range 11.8–32 kg). A 4F catheter was used for delivery of the coils in all patients. Three patients underwent multiple coil and two single coil closure with complete elimination of the shunt. There was complete elimination of the shunt as documented by repeat angiography immediately after the coil closure. The median fluoroscopy time was 24 min (range, 22–55 min). All patients were discharged home on the same day. There were no complications Follow-up evaluation was performed with color flow mapping of the main pulmonary artery within 4 hr after closure and 6 weeks later with echocardiography and chest radiography. We conclude that transcatheter closure is an effective therapy for patients with residual PDA after the Rashkind device using the single or multiple coil technique. This technique can be performed on an out-patient basis without the need for general anesthesia and at a lower expense than a repeat occluder device or surgery. © 1995 Wiley-Liss, Inc.

Surgical repair for recurrent or residual postoperative coarctation of the aorta is associated with some morbidity, mortality, and an incidence of recurrent coarctation that may be as high as 20%. Balloon angioplasty for recurrent or residual postoperative coarctation has become the standard treatment of choice for this condition. In this article, we review the protocol we use, and the acute and long-term results of this procedure. Since 1983, over 400 patients have undergone balloon angioplasty for recurrent coarctation with excellent immediate relief of the gradient and improvement in the diameter. On long-term follow-up, the majority of these patients enjoyed a lasting beneficial effect of the balloon angioplasty. We discuss the future use of stents for selected patients who are not amenable or failed balloon angioplasty of recurrent or residual postoperative coarctation.

Background Chest pain is a common reason for referral to pediatric cardiologists. Although pediatric chest pain is rarely attributable to serious cardiac pathology, extensive and costly evaluation is often performed. We have implemented a standardized approach to pediatric chest pain in our pediatric cardiology clinics as part of a broader quality improvement initiative termed Standardized Clinical Assessment and Management Plans (SCAMPs). In this study, we evaluate the impact of a SCAMP for chest pain on practice variation and resource utilization. Methods and Results We compared demographic variables, clinical characteristics, and cardiac testing in a historical cohort (n=406) of patients presenting to our outpatient division for initial evaluation of chest pain in the most recent pre‐SCAMP calendar year (2009) to patients enrolled in the chest pain SCAMP (n=364). Demographic variables including age at presentation, sex, and clinical characteristics were similar between groups. Adherence to the SCAMP algorithm for echocardiography was 84%. Practice variation decreased significantly after implementation of the SCAMP ( P P ‐enrolled patients compared with the historic cohort (~3% of patients versus 29%, respectively; P P =0.003 and P Conclusions Implementation of a SCAMP for evaluation of pediatric chest pain has lead to a decrease in practice variation and resource utilization. ( J Am Heart Assoc . 2012;1:jah3‐e000349 doi : 10.1161/JAHA.111.000349 .)

Retrograde coil embolization of the patent ductus arteriosus (PDA) has been reported for ducts3.3 mm in diameter using 5 to 5.5Fr catheters. Our objective was to assess the immediate and short-term results of anterograde transcatheter closure of the PDA using single or multiple Gianturco coils. Thirty-three patients (11 males and 22 females) underwent an attempt at transcatheter closure of the PDA anterogradely at a median age of 2.8 years (range 0.1 to 19) and median weight of 14 kg (range 2.3 to 64). The median PDA diameter at its narrowest segment was 2.8 mm (range 0.5 to 5.3). A 4Fr catheter was used for delivery of the coils in 30 patients and a 3Fr catheter was used in 3 patients. Eighteen patients underwent multiple coil closure for a PDA diameter ofor = 2.5 mm (using 2 coils in 9, 3 coils in 6, 4 coils in 1, and 5 coils in 2 patients). Follow-up evaluation was performed with color flow mapping of the main pulmonary artery. Thirty-one of the 33 patients had complete closure by the coil technique. The 2 unsuccessful attempts were in 2 patients with a large ductus measuringor = 4.3 mm and of angiographic type B. For all 13 patients in whom a single coil and in 17 patients in whom multiple coils were placed, there was immediate complete closure that persisted at a median follow-up period of 3.8 months. The median fluoroscopy time was 24 minutes.(ABSTRACT TRUNCATED AT 250 WORDS)

OBJECTIVES: Chest pain is a common reason for referral to pediatric cardiologists and often leads to an extensive cardiac evaluation. The objective of this study is to describe current management practices in the assessment of pediatric chest pain and to determine whether a standardized care approach could reduce unnecessary testing. PATIENTS AND METHODS: We reviewed all patients, aged 7 to 21 years, presenting to our outpatient pediatric cardiology division in 2009 for evaluation of chest pain. Demographics, clinical characteristics, patient outcomes, and resource use were analyzed. RESULTS: Testing included electrocardiography (ECG) in all 406 patients, echocardiography in 175 (43%), exercise stress testing in 114 (28%), event monitoring in 40 (10%), and Holter monitoring in 30 (7%). A total of 44 (11%) patients had a clinically significant medical or family history, an abnormal cardiac examination, and/or an abnormal ECG. Exertional chest pain was present in 150 (37%) patients. In the entire cohort, a cardiac etiology for chest pain was found in only 5 of 406 (1.2%) patients. Two patients had pericarditits, and 3 had arrhythmias. We developed an algorithm using pertinent history, physical examination, and ECG findings to suggest when additional testing is indicated. Applying the algorithm to this cohort could lead to an ∼20% reduction in echocardiogram and outpatient rhythm monitor use and elimination of exercise stress testing while still capturing all cardiac diagnoses. CONCLUSIONS: Evaluation of pediatric chest pain is often extensive and rarely yields a cardiac etiology. Practice variation and unnecessary resource use remain concerns. Targeted testing can reduce resource use and lead to more cost-effective care.

Presentation of Case A 48-year-old woman was admitted to the hospital because of a secundum atrial septal defect with severe pulmonary hypertension. A murmur was first heard when the patient was 11 years of age. At 21 years she began to experience palpitations during exercise and had auscultatory findings characteristic of an atrial septal defect. Eight years later she was dyspneic when climbing three flights of stairs and had frequent respiratory tract infections. She was seen at this hospital, and the risk of a cardiac surgical procedure was considered unacceptable. At 39 years of age supraventricular tachycardia occurred and responded . . .

Chest pain in children is common, but rarely heralds serious underlying cardiac pathology. Despite this, the anxiety of missing a potentially life threatening condition creates a large burden of referrals and diagnostic testing. We evaluated patients diagnosed with 1 of 9 serious cardiac diseases and detailed the clinical signs and symptoms of the patients presenting with chest pain.Patients diagnosed between the ages of 7 and 21 years from January 2000 to December 2009 at Children's Hospital Boston (CHB) were identified from a database using diagnostic and billing codes for aortic dissection, coronary anomalies, dilated cardiomyopathy, hypertrophic cardiomyopathy, myocarditis, pericarditis, pulmonary embolus, pulmonary hypertension, and Takayasu arteritis. Patients with previously diagnosed congenital or acquired heart disease were excluded.Four hundred eighty-four patients were included and 35% presented with chest pain. Forty-one (24%) of these patients with chest pain were diagnosed in the outpatient cardiology clinic, while the remaining 130 patients (76%) were diagnosed in the emergency department (ED) or inpatient setting. Coronary artery anomalies were the most common diagnosis made in cardiology clinic, and 16 of the 23 (70%) patients with serious coronary anomalies had exercise-induced chest pain. Patients presenting to the ED or inpatient units tended to have other important nonspecific symptoms (35-44%), high-risk past medical histories (12%), physical examination findings (32%), and electrocardiogram (ECG) abnormalities (78%) that heighten clinical suspicion of cardiac disease.Identifying underlying cardiac pathology in the CHB outpatient cardiology department in patients presenting with chest pain is rare, with only 41 cases over a 10-year period. The presence of exertional chest pain was important in identifying patients with coronary artery anomalies. A detailed history and physical examination, along with a critical review of an ECG, seem to identify those patients with rare diseases who need further diagnostic testing.

We report 4 teenage patients who, after successful palliation of severe aortic valve stenosis by balloon aortic valvuloplasty in early infancy, presented within the previous 3 years with significant left ventricular (LV) diastolic heart failure. All patients had remained asymptomatic until their teenage years and had normal or hyperdynamic LV systolic function on presentation, with limited residual aortic valve stenosis and regurgitation. All underwent echocardiography, cardiac catheterization, and cardiac magnetic resonance evaluation. One notable common feature in the 4 patients was the presence of a confluent layer of LV subendocardial hyperenhancement demonstrated by late gadolinium enhancement technique. Histopathology was available for 2 patients, which documented significant LV endocardial fibroelastosis. One patient who underwent endocardial fibroelastosis resection in conjunction with aortic valve replacement had late clinical improvement. In conclusion, this group of patients may represent an important emerging clinical entity and merits close clinical surveillance, with prospective assessment of diastolic function.

Fifty-six patients undergoing a modified Fontan procedure from 1977 to 1985 had lung tissue available from the perioperative period (n = 51) or recent palliative procedure (n = 5) (37 biopsy, 12 autopsy, 7 biopsy and autopsy). Twenty-six patients had tricuspid atresia, and 30 had other causes of functional univentricular heart. Pulmonary arterial structure was classified by both the Heath-Edwards and morphometric scales. Biopsy and autopsy grades were identical except for a minor difference in Heath-Edwards scale in one case. Elevated preoperative mean pulmonary artery pressure (>15 mm Hg) was associated with medial hypertrophy (p = 0.04), although six patients with normal pulmonary artery pressure had medial hypertrophy and six patients with mean pulmonary artery pressure greater than 15 mm Hg did not have medial hypertrophy. Medial hypertrophy was a possible risk factor in patients with univentricular heart: Patients with medial hypertrophy had a ninefold greater risk of death (p = 0.08). Controlling for confounding variables did not alter this result (0.06

Data from exercise tests, echocardiograms, and lung perfusion scans were analyzed to determine whether the excessive minute ventilation (VE) often encountered among patients with tetralogy of Fallot is due to ventilation-perfusion mismatch secondary to branch pulmonary artery stenoses. Patients with branch PA stenoses had lower peak oxygen consumptions and higher VE during exercise than did patients without stenoses, and a strong correlation existed between the degree of pulmonary blood flow maldistribution on lung perfusion scan and the amount of excessive VE during exercise.

Catheter-induced ventricular ectopy can limit assessment of contractility or anatomy. We compared the incidence of ventricular ectopy in infants and children undergoing left ventriculography using a 4F Halo catheter or a 4F pigtail catheter. For each group, 17 patients had 19 ventriculograms. Iopamidol 76% was used for each study. There was no statistical difference between the Halo and pigtail groups (mean ± SEM) for age (16.2 ± 3.8 vs. 12.9 ± 1.8 mo), weight (8.1 ± 1.0 vs. 8.0 ± 0.7 kg), gender, left ventricular end diastolic pressure (9.6 ± 1.1 vs. 9.8 ± 1.3 mm Hg), catheter location in the ventricle, or volume of contrast (1.31 ± 0.06 vs. 1.35 ± 0.06 cc/kg). The Halo group had a more rapid injection rate (1.42 ± 0.15 vs. 0.99 ± 0.10 cc/kg/sec, P 1 beat (1 vs. 10 studies, P < 0.002), and ventricular couplets or tachycardia (1 vs. 9 studies, P < 0.008). The 4F Halo catheter is associated with less ventricular ectopy than a 4F pigtail catheter and should be considered for use during pediatric catheterization. Cathet. Cardiovasc. Diagn. 43:55–57, 1998. © 1998 Wiley-Liss, Inc.

Background: The improved survival of very low-birth-weight (

The purpose of this research was to investigate the causes and symptoms of superior vena cava (SVC) obstruction or occlusion and report on the long-term results of transcatheter therapy.Information on transcatheter therapy for SVC obstruction is limited.Superior vena cava catheterization interventions between August 1984 and April 2006 were reviewed. Patients were divided into 2 subgroups depending on whether or not they had previously undergone congenital cardiac surgery.Sixty-three patients with median age of 3.7 years (range 1 month to 42 years) and weight of 13.3 kg (range 3 to 114 kg) were treated. Fifty patients (79%) were symptomatic, although only 50% had symptoms suggestive of SVC obstruction. Superior vena cava syndrome was more common in the non-cardiac surgical group (52% vs. 10%, p = 0.001). The mean gradient and SVC diameter improved from 10.8 +/- 5.8 mm Hg to 2.6 +/- 2.2 mm Hg (p0.001) and 3.1 +/- 2.7 mm to 9.1 +/- 3.8 mm, respectively (p0.001). The obstruction was adequately relieved in all 36 patients receiving stents and in 21 of 27 patients (78%) who had balloon dilation alone. Complications occurred in 12 patients (19%), all of whom had previously undergone cardiac surgery; 10 of these patients were successfully treated in the catheterization laboratory. Freedom from re-intervention did not differ between patients undergoing balloon dilation or stent implantation, but was longer in patients age5 years at the time of intervention.Superior vena cava-related symptoms occur in only 50% of patients with hemodynamically significant SVC obstruction. Endovascular therapy is successful in relieving the stenosis and associated symptoms with good long-term results.

We describe two brothers with congenital lymphedema of lower limbs, atrial septal defect (ASD), and similar facial appearance. A sister had severe hydrops fetalis, ASD, omphalocele, and other anomalies. This combination of congenital lymphedema and ASD differs from other reported cases of congenital lymphedema and most likely constitutes a previously unrecognized autosomal recessive syndrome.

A 61-year-old woman with levo-transposition of the great arteries, double-inlet single left ventricle, and valvar and subvalvar pulmonary stenosis presented with a large pulmonary valve vegetation unresponsive to antibiotic therapy. The diagnostic evaluation and the surgical management are discussed. At operation the pulmonary valve was excised and an abscess cavity was obliterated with a pericardial patch. She is currently doing well 3 years after the operation.

Objective. To determine the basis for cardiac consultations for pediatric patients in an academic hospital setting.Methods. The activities of the cardiology consultation service were tabulated for 12 months, from July 2001 to June 2002. Patients were identified from 4 sources, ie, a monthly log of patient encounters maintained by the consultation service, encounter forms submitted to the billing office, consultation notes maintained in a central file, and a departmental list of echocardiography studies. Patients who required clearance for noncardiac surgical procedures were generally evaluated in the cardiology clinic and not by the consultation service. Patient data were obtained from consultation and echocardiography reports and from hospital computer-based records for discharge summaries for inpatient admissions, emergency department encounter summaries, and laboratory reports. For each patient, consultations were tabulated as separate encounters if they occurred on different days in the emergency department, during separate admissions, or for different clinical concerns during a single admission.Results. A total of 2071 consultations were performed for 1724 patients. The age at the time of consultation was 6.6 ± 9.3 years (median: 1.2 years; range: 1 day to 60.6 years). A total of 1507 patients (87.4%) had a single consultation; 217 patients (12.6%) had multiple encounters, ranging from 2 to 9, accounting for 564 consultations (27.2%). Clinical concerns included murmurs (18.5%), cardiac function (12.7%), arrhythmias (12.7%), intercurrent illnesses among cardiac patients (11.3%), cyanosis (6.3%), syndromes (5.7%), chest pain (5.2%), syncope/dizziness (4.5%), subacute endocarditis (4.4%), follow-up evaluations of fetal diagnoses (4.3%), Kawasaki disease (3.4%), cor pulmonale (3%), recent cardiac surgery or catheterization (1.6%), cerebrovascular accidents (1.2%), and miscellaneous conditions. Four diagnoses accounted for 91% of murmur evaluations, ie, patent ductus arteriosus, ventricular septal defects, innocent murmurs, and pulmonary branch murmur of infancy. The most common murmur diagnosis in the neonatal intensive care unit was patent ductus arteriosus (68%), in the well-child nursery was ventricular septal defect (64%), and on the medical ward was innocent murmur (62%). The most common basis for evaluation of function was oncologic disease. Among patients evaluated for function, there were 3 new diagnoses of structural congenital heart disease, all involving neonates with aortic arch obstruction. Approximately two-thirds of arrhythmias were supraventricular in origin. The most common arrhythmias requiring treatment were supraventricular tachycardia and atrial flutter/fibrillation, the latter occurring mainly among older patients with structural heart disease. Diagnoses made with fetal echocardiography accounted for 14.3% of newborn consultations and included 83% of patients with cyanotic cardiac disease. Three syndromes accounted for 57% of consultations for this indication, ie, VACTERL association (vertebral anomalies, anal atresia, congenital heart disease, tracheoesophageal fistula, renal abnormality, and limb anomalies), trisomy 21, and infant of diabetic mother. Chest pain and syncope/dizziness were frequently evaluated in the emergency department and, in this setting, accounted for 13 and 10% of all evaluations and 19 and 25% of evaluations for new patients, respectively. For patients evaluated for chest pain, the most common basis was musculoskeletal/costochondritic (42%) or idiopathic (22%). There was a cardiac or pericardial basis in 11% of cases; these patients either had known heart disease associated with this complication or systemic symptoms, abnormal cardiac auscultatory findings, and electrocardiographic features of pericarditis. Syncope/dizziness most commonly had a vasovagal (50.5%) or orthostatic (24.7%) basis. There was a cardiac basis in 5.4% of cases; these patients were more likely to have symptoms associated with exercise. Although endocarditis was a frequent clinical concern (91 patients), only 3 cases were identified, involving 2 patients with structural heart disease and 1 neonate with an indwelling intracardiac catheter. Two other patients had central venous lines, intravascular thrombus, and fungemia. Kawasaki disease was the most common acquired condition leading to consultation. Cor pulmonale was most commonly screened among patients with congenital diaphragmatic hernia, chronic lung disease of prematurity, pneumonitis, reactive airway disease, or cystic fibrosis. Patients with recent cardiac surgery or cardiac catheterization typically had postpericardiotomy syndrome or complications associated with vascular access. Approximately 20% of cases of cerebrovascular accidents had a cardiac basis.Conclusions. Although a variety of conditions were assessed, some were encountered more frequently. Future educational curricula developed for cardiac training of pediatric residents should appropriately emphasize conditions necessitating consultation.

There is limited reported experience of catheterization therapy for peripheral pulmonary stenosis (PPS) at a surgical site in the early postoperative period. We reviewed the clinical course of patients undergoing interventional catheterization for PPS at a surgical site 50% increase in predilation diameter. From 1984 to 2000, 17 patients had 19 proximal pulmonary arteries dilated 1 to 46 (median 8) days postoperatively. Median age and weight were 3.1 year and 12.7 kg. Three arteries were initially occluded. Seventeen arteries had initial BD with postintervention imaging available in 15; 8 arteries had SD. The arterial diameter increased from 3.9 ± 2.6 to 5.5 ± 2.8 mm (P < 0.001). Nine of these arteries had stents placed with diameter increasing to 8.7 ± 3.7 mm (P < 0.001 compared with post-BD diameter). Stents increased the diameter in all arteries and made four of four failed BD successful. In the two most recent procedures, stents were placed without prior BD with diameter increasing from 1.3 to 9 mm and 8.2 to 14 mm. A stent was placed in 1 of 7 arteries prior to 1993 and in 10 of 12 arteries thereafter (P < 0.004). Three patients prior to 1995 had catheterization-related deaths secondary to vessel rupture after BD. BD produces SD in approximately one-half of the procedures but is associated with mortality. Stent placement increases vessel diameter substantially more than BD alone. Stents reduce the acute complication rate and avoid early reoperation in this patient group. Cathet Cardiovasc Intervent 2002;56:272–277. © 2002 Wiley-Liss, Inc.

Objective: We assessed factors contributing to parental anxiety when children are referred to a cardiology clinic for evaluation of a Still's murmur. Methods: Parents of 95 children completed questionnaires designed to assess family and patient characteristics, parents' ratings of their anxiety and the reassurance they received from their pediatrician, and current (state) and general anxiety levels. Results: Parents reported anxiety about multiple issues including the need for medication (49%), sports restrictions (41%), cardiac surgery (29%), cardiac risk for siblings (20%), and premature death (13%). Of reporting mothers, 19% felt the murmur resulted from something they did wrong during pregnancy. Although 54% of parents were extremely reassured by their pediatrician, only 17% had no anxiety associated with the specialty visit. After reassurance from the cardiologist, 7% of parents had persistent anxiety. In multivariable analysis, 2 features, both related to the referring pediatrician, were significantly related to parental anxiety level. High parental anxiety was associated with lower pediatrician reassurance ratings and greater pediatrician practice years. Conclusions: Parental anxiety is common among parents of children referred for specialty evaluation. Educational strategies to improve pediatrician communication skills with parents may improve quality of care. (J Pediatr 2002;140:747-52)

Background —Experience of balloon dilation of peripheral pulmonary stenosis (PPS) in Williams syndrome (WS) is limited. Methods and Results —Catheterizations in all patients with WS undergoing therapy for PPS from 1984 to 1999 were reviewed. Criteria for successful dilation included an increase >50% in predilation diameter and a decrease >20% in ratio of right ventricular (RV) to aortic (Ao) systolic pressure. Median age and weight were 1.5 years and 9.5 kg. There were 134 dilations during 39 procedures in 25 patients. The success rate for initial dilations was 51%. In multivariate analysis, successful dilation was more likely (1) in distal than in central pulmonary arteries ( P =0.02), (2) if the balloon waist resolved with inflation ( P =0.001), and (3) with larger balloon/stenosis ratio ( P P =0.03), and the RV/Ao pressure ratio decreased (0.97±0.34 versus 0.91±0.30, P =0.05). Aneurysms developed after 24 dilations (18%) and were not related to balloon/stenosis ratio. Balloon rupture in 12 dilations produced an aneurysm in all 7 cases when rupture was in a hypoplastic segment. Three patients died, none from pulmonary artery trauma, and all before 1994. Conclusions —Mortality occurred early in our experience. Despite successful dilation of distal pulmonary arteries, there was modest initial hemodynamic improvement, mainly because of persistent central pulmonary artery obstruction. A serial approach of distal dilations followed by surgical repair of proximal obstruction may be a rational and successful therapy.

Background . Recent surgical reports on coarctation of the aorta have primarily focused on the relative merits of various operative techniques. However, appropriate timing for elective repair remains unclear. Methods . In a retrospective analysis we examined the surgical outcomes in 176 consecutive patients undergoing repair of coarctation of the aorta in our institution over a 25-year period. Ninety-nine percent of the patients had follow-up for a median of 7.5 years. Results . A total of 13 patients have died (7.4% overall mortality). Nine of these patients had associated complex intracardiac anomalies. There was no mortality in the 113 patients with isolated coarctation. Residual or recurrent coarctation occurred in 27 patients (15.3%). The age at operation and the type of surgical repair did not have an effect on the incidence of recurrence. Persistent or late hypertension was identified in 18 of the 107 patients who have been followed up for more than 5 years (16.8%). A total of 48 patients operated on during infancy have been followed up for more than 5 years. Only 2 have developed late hypertension (4.2%). Both of these patients had recurrence. In contrast, 16 of the 59 patients operated on after a year of age had late hypertension (27.1%). Conclusions . To minimize the risk of persistent hypertension, elective repair of coarctation should be performed within the first year of life.

To determine whether survivors of surgery for coarctation of the aorta (CoA) have an excessive reliance on anaerobic metabolism during exercise.Patients with peripheral vascular disease cannot increase blood flow to their muscles normally during exercise. Consequently they acquire an early, excessive reliance on anaerobic metabolism and have depression of the ventilatory anaerobic threshold (VAT) and of the slope of the oxygen consumption-work rate relationship (delta VO2/delta WR). We speculated that the capacity to augment blood flow to the lower extremities during exercise may be impaired after CoA surgery and would result in similar metabolic disturbances.Progressive exercise tests were performed on 15 patients (ages 19 +/- 7 years; range, 10 to 32) after successful repair of CoA (residual resting gradient, 7.7 +/- 7.1 mm Hg; range, 0 to 18), 15 age- and sex-matched healthy control subjects, and 10 patients (ages 13 +/- 3 years; range, 10 to 20) who had undergone ligation of a patent ductus arteriosus.The CoA patients' VAT averaged 14.8 +/- 3.8 ml O2/kg per minute versus 19.3 +/- 3.1 ml O2/kg per minute for the control subjects (p0.01), and their delta VO2/delta WR averaged 8.2 +/- 1.8 ml/watt compared with 10.1 +/- 1.4 ml/watt for control subjects (p0.01). Furthermore, 10 of 15 CoA patients had a VAT of less than 40% of predicted maximal oxygen consumption, and 9 of 16 had a delta VO2/delta WR of less than 8.7 ml O2/watt (generally accepted abnormal values). Patients with patent ductus arteriosus resembled the healthy control subjects with regard to anaerobic metabolism during exercise.Patients who have had CoA repairs commonly manifest an excessive reliance on anaerobic metabolism during exercise. This phenomenon may result from persistent blood flow abnormalities across the aortic arch during exercise, which may be present even after apparently successful surgery.