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BACKGROUND Fetal echocardiography can accurately diagnose critical congenital heart disease prenatally, but relies on referrals from abnormalities identified on routine obstetrical ultrasounds. Critical congenital heart disease that is frequently missed due to inadequate outflow tract imaging includes anomalies such as truncus arteriosus, double outlet right ventricle, transposition of the great arteries, tetralogy of Fallot, pulmonary stenosis, and aortic stenosis. OBJECTIVE This study evaluated the prenatal detection rate of critical outflow tract anomalies in a single urban pediatric hospital before and after "AIUM Practice Guideline for the Performance of Obstetric Ultrasound Examinations," which incorporated outflow tract imaging. DESIGN Infants with outflow tract anomalies who required cardiac catheterization and/or surgical procedure(s) in the first 3 months of life were retrospectively identified. This study evaluated two time periods; pre-guidelines from June 2010 to May 2013 and post-guidelines from January 2015 to June 2016. June 2013-December 2014 was excluded as a theoretical period necessary for obstetrical practices to implement the revised guidelines. RESULTS Overall, prenatal diagnosis occurred in 55% of infants with critical outflow tract anomalies; of the three most common defects, prenatal diagnosis occurred in 53% of D-transposition of the great arteries, 63% of tetralogy of Fallot, and 80% of double outlet right ventricle patients. Pre-guidelines, prenatal diagnosis occurred in 52% (52 of 102) infants with critical outflow tract anomalies requiring early cardiac intervention. Post-guidelines, prenatal diagnosis occurred in 61% (33 of 54) infants, not significantly different than the prenatal detection rate pre-guidelines (P = .31). CONCLUSIONS Despite revised obstetrical guidelines highlighting the importance of outflow tract imaging, referrals and prenatal diagnosis of these types of critical congenital heart disease remain low. Education of obstetrical sonographers and practitioners who perform fetal anatomic screening is vital to increase referrals and prenatal detection of critical outflow tract anomalies.

ObjectivePatients with congenital heart disease may be operated with surgical reconstruction of the right ventricular outflow tract (RVOT). Reintervention is common in this group of patients. The aim of this study was to examine longitudinal self-reported health measured by the EQ-5D questionnaire. DesignData were collected from the Swedish Registry of Congenital Heart Disease. EQ-5D, which measures health outcome, was introduced into the registry in 2005. All adult patients with previous surgical reconstruction of the RVOT who had EQ-5D data from their first and latest visit were analyzed. ResultsAmong 103 patients (65 male and 38 female), mean age 31 (range 19-78 years), the diagnoses were: tetralogy of Fallot (n=66); truncus, transpositions, and double outlet right ventricle (n=23); and Ross-operated congenital aortic valve disease (n=14). Time from first to latest visit was 3 years (range 1-7 years). Eighteen patients underwent 26 reinterventions in the observational period from the first to latest visit, including operations, percutaneous interventions, pacemaker implantations, and ablations. Health perception, mean EQ-5D visual analog scale, VAS, declined from 84.4 (standard deviation (SD)=14.6) to 78.6 (SD=18.3) at the latest visit, P=.001. The decline is almost exclusively seen in patients without reinterventions. Low EQ-VAS was associated with symptoms and New York Heart Association class II-IV. Patient-reported problems in the EQ-5D dimension usual activities were more common in the patients having reinterventions (25%) than those without reintervention (7%), P=.04. ConclusionIn this longitudinal cohort study of patients with previous surgical reconstruction of the RVOT, health perception declined over time. The decline was not observed in patients undergoing any additional interventions.

A 52-year-old male patient, with a medical history of surgically repaired double outlet right ventricle presented with severe aortic stenosis (AS) and hepatitis C with cirrhosis, presented with New York Heart Association Class IV heart failure. During evaluation for a liver transplant, he was deemed a poor surgical candidate due to his aortic valve disease and cirrhosis with model for end-stage liver disease score of 14. Transthoracic echocardiogram showed severe AS with a mean gradient of 62 mm Hg and calculated aortic valve area of 0.74 cm(2) with a normal ejection fraction of 65%. The patient underwent transfemoral implantation of a 23-mm Edwards Sapien commercial heart valve with significant mean gradient reduction across the aortic valve from 62 to 13 mm Hg. The patient was observed in the coronary care unit and discharged home 2 days postprocedure with his clinical symptoms greatly improved.

Absence of the aortic valve is a rare congenital heart defect that is detectable in the prenatal period. In this condition, functional aortic valve leaflets are absent; in their place are rudimentary, immobile noncoapting plate-like structures at the level of the annulus resulting in severe aortic regurgitation. We report the fetal diagnosis and subsequent postnatal course of an infant with the novel association of absent aortic valve, double outlet right ventricle, and aortopulmonary window. This unique combination of defects resulted in an unusual pattern of circular shunting that produced evidence of fetal heart failure. Shortly after birth, the abnormal physiology led to compromised systemic perfusion, intestinal perforation and subsequent rapid demise of the patient. Abrupt postnatal deterioration typifies the vast majority of the absent aortic valve cases found in the literature. Our patient is unique in that it is the first female reported with absent aortic valve and the first reported with an aortopulmonary window. This report demonstrates that absent aortic valve is a condition that can be diagnosed prenatally by fetal echocardiogram; the highly abnormal physiology places these patients at risk for fetal heart failure. Given the potential for clinical instability and rapid deterioration after birth, such patients should undergo rapid postnatal assessment and immediate surgical intervention when deemed appropriate.

Objective We evaluated left ventricular dimensions and aortic arch z-scores in infants who underwent balloon angioplasty (BAP) or surgery for coarctation of aorta (CoA). We searched for risk factors predicting recoarctation. Patients Between 2007–2011, 27 male and 17 female infants (mean age 2.93 ± 4.78 months, range 2 days–24 months) with CoA were evaluated. Left ventricular dimensions, systolic functions, mitral and aortic annuli, transverse aortic arch, isthmus, coarctation site, and diaphragmatic aorta measurements were done and z-scores were determined before intervention. Results Six patients underwent primary operation, 38 patients had BAP (86.4%). Associated cardiac pathologies in operated patients were double outlet right ventricle (n = 2), atrioventricular septal defect (n = 1), Ebstein's anomaly (n = 1), arch hypoplasia (n = 2). Twelve patients (27.2%) had simple coarctation. Ventricular septal defect was the most frequent associated cardiac pathology (n = 20, 45.4%). The patients were followed for 10.22 ± 8.21 months. Among 33 primary successful BAP's, 14 had recoarctation (42%). Eleven patients were primarily operated (including 5 with unsuccessful BAP), two had recoarctation (18%). Abdominal and transverse aorta values and z-scores were significantly lower in the recoarctation group (7.15 ± 2.12 mm and 6.07 ± 1.86 mm respectively in the “no-recoarctation group”; vs. 5.53 ± 0.75 mm and 4.94 ± 1.53 mm in the “recoarctation group” P

Double outlet right ventricle is a heterogeneous congenital defect that encompasses a variety of anatomic aberrations and physiologic consequences. We describe the unusual cardiovascular magnetic resonance anatomic findings and sequelae of a 44-year-old man who underwent biventricular repair of double outlet right ventricle, subpulmonary type, which included tunneling of the ventricular septal defect to the pulmonary artery, right ventricular-to-pulmonary artery conduit and Damus-Kaye-Stansel procedure.

Purpose The aim of this article was to assess the role of MRI in the diagnosis and management of a variety of complex aortic arch anomalies. Materials and methods Imaging was performed on a 1.5T Philips Gyroscan Intera. We retrospectively reviewed all cardiac MR scans performed from November 2003 to February 2007 at our institute to identify aortic arch anomalies. Magnetic resonance imaging included three-dimensional (3D) sagittal volume images, cine images, short axis ventricular volumes, phase contrast flow of great vessels, and 3D gadolinium magnetic resonance angiogram. Results Three major categories of anomalies were identified in 47 patients: double aortic arch (n = 9), right-sided aortic arch with mirror image branching (n = 28), and aberrant right subclavian artery (n = 10). In the double aortic arch group, six patients were symptomatic, and four of them underwent a surgical division. Magnetic resonance imaging was used to plan the surgical management. In the other two groups, no patient presented with symptoms of airway or esophageal compression, and the arch abnormalities were noticed during investigation for other associated cardiac disease. However, we noticed a strong correlation with well-defined subgroups of congenital heart disease. Right-sided aortic arch was seen in patients with pulmonary atresia with ventricular septal defect (46.4%), tetralogy of Fallot (32.1%), and double outlet right ventricle with right atrial isomerism (14.2%). Seventy percent of the patients with aberrant right subclavian artery had aortic coarctation, and another case presented a complete aortic arch interruption. Conclusion Magnetic resonance imaging is an important diagnostic tool in identifying anomalies of the aortic arch and its branches and can be considered the imaging technique of choice when planning surgical management, especially when there are associated cardiac anomalies.

Objective. Double-outlet right ventricle (DORV) is a complex congenital heart defect with heterogeneous anatomy. Patients require a variety of surgical interventions, and the long-term prognosis is variable. Therefore, accurate prenatal diagnosis is important in providing parents with appropriate counseling. Design. Medical records were reviewed in patients with a diagnosis of DORV who had fetal echocardiography at our institution from 1998 to 2004. Pre- and postnatal diagnoses were compared, anticipated surgical procedure was compared with surgery performed, and neonatal outcome was assessed. Results. The study group consisted of 49 fetal patients with 6 in utero deaths (including 4 terminations), 2 patients lost to follow-up, and 41 live births. Postnatal echocardiograms or autopsy results were available on 42 patients. The overall accuracy of fetal echocardiography in making a correct diagnosis of DORV was 76%. Accurate prenatal prediction of the type of cardiac surgery that would be performed was made in 91% of patients who had surgery. Among live births, survival to hospital discharge was 76%. Survival was 50% in patients with extracardiac or chromosomal anomalies, compared with 92% in infants without additional anomalies. Conclusion. In most cases, careful evaluation by fetal echocardiography can determine essential anatomic details in fetuses with DORV with enough accuracy to allow for accurate counseling regarding the type of surgery needed. Survival is better than previously reported but is poor in patients with extracardiac or chromosomal abnormalities.

A 52-year-old male patient, with a medical history of surgically repaired double outlet right ventricle presented with severe aortic stenosis (AS) and hepatitis C with cirrhosis, presented with New York Heart Association Class IV heart failure. During evaluation for a liver transplant, he was deemed a poor surgical candidate due to his aortic valve disease and cirrhosis with model for end-stage liver disease score of 14. Transthoracic echocardiogram showed severe AS with a mean gradient of 62 mm Hg and calculated aortic valve area of 0.74 cm(2) with a normal ejection fraction of 65%. The patient underwent transfemoral implantation of a 23-mm Edwards Sapien commercial heart valve with significant mean gradient reduction across the aortic valve from 62 to 13 mm Hg. The patient was observed in the coronary care unit and discharged home 2 days postprocedure with his clinical symptoms greatly improved.

Absence of the aortic valve is a rare congenital heart defect that is detectable in the prenatal period. In this condition, functional aortic valve leaflets are absent; in their place are rudimentary, immobile noncoapting plate-like structures at the level of the annulus resulting in severe aortic regurgitation. We report the fetal diagnosis and subsequent postnatal course of an infant with the novel association of absent aortic valve, double outlet right ventricle, and aortopulmonary window. This unique combination of defects resulted in an unusual pattern of circular shunting that produced evidence of fetal heart failure. Shortly after birth, the abnormal physiology led to compromised systemic perfusion, intestinal perforation and subsequent rapid demise of the patient. Abrupt postnatal deterioration typifies the vast majority of the absent aortic valve cases found in the literature. Our patient is unique in that it is the first female reported with absent aortic valve and the first reported with an aortopulmonary window. This report demonstrates that absent aortic valve is a condition that can be diagnosed prenatally by fetal echocardiogram; the highly abnormal physiology places these patients at risk for fetal heart failure. Given the potential for clinical instability and rapid deterioration after birth, such patients should undergo rapid postnatal assessment and immediate surgical intervention when deemed appropriate.

Crisscross heart is a rare congenital cardiac anomaly in which systemic and pulmonary venous streams cross without mixing at atrioventricular level. We report a case of crisscross heart with tricuspid atresia, double outlet right ventricle, and pulmonary stenosis, which was diagnosed prenatally.

Double outlet right ventricle is a heterogeneous congenital defect that encompasses a variety of anatomic aberrations and physiologic consequences. We describe the unusual cardiovascular magnetic resonance anatomic findings and sequelae of a 44-year-old man who underwent biventricular repair of double outlet right ventricle, subpulmonary type, which included tunneling of the ventricular septal defect to the pulmonary artery, right ventricular-to-pulmonary artery conduit and Damus-Kaye-Stansel procedure.

To characterize the pharmacotherapeutic regimens used in infants with single ventricle heart disease and determine the influence of outpatient medications on interstage weight gain.Retrospective review.Tertiary care pediatric hospital.All patients discharged from our institution with single ventricle heart disease that underwent neonatal first stage surgical palliation between 2002 and 2009 were included. Patients who died prior to second stage palliation or underwent orthotopic heart transplantation were excluded.Outpatient medication regimens during the interstage period were reviewed. Medication regimens were compared between surgical eras and between patient groups experiencing different outcomes. A logistic regression model was developed to determine independent factors for an interstage increase in weight-for-age z-score (WAZ) and a linear regression model to determine medications significant for an increase in weight gain per day.The study cohort consisted of 161 patients (58% male). Most patients in this cohort had either hypoplastic left heart syndrome (51%) or unbalanced complete atrioventricular canal (29%). Patients were placed on a median of four medications (range 1-9) at discharge from first surgical palliation, with aspirin (79%), furosemide (79%), and angiotensin converting enzyme inhibitors (ACE-I) (73%) most commonly prescribed. A median of six medication doses per day (range 2-18) were prescribed at discharge. Most patients (71%) had a decrease in WAZ during the interstage period. Use of digoxin (P0.01) and high-dose furosemide (P = .02) were associated with a decrease in WAZ score during the interstage period. Additionally, the use of ACE-I, ranitidine, proton-pump inhibitors, or promotility agents was not associated with improved somatic growth during the interstage period.Infants with single ventricle heart disease have a high-medication burden during the interstage period. Despite the focused and intensified use of medications to improve feeding tolerance and somatic growth, current pharmacotherapeutic regimens appear to have little effect on interstage weight gain.

Surgical alternatives to biventricular repair of complex forms of double outlet right ventricle (DORV) remain controversial. The available knowledge of the Fontan operation (FO) for children with this anomaly is limited. The aim of this report is to analyze the results and risk factors of the FO performed as an alternative to the biventricular repair of complex DORV.Between 1980 and 2007, 47 patients with complex forms of DORV underwent a modified FO. Concomitant defects included noncommitted, subpulmonary or multiple ventricular septal defects, anomalies of atrioventricular valves and/or caval veins, and ventricular hypoplasia. Thirty-five of 45 patients (77%) have undergone a hemi-Fontan and 35 patients (77%; 10 children without previous hemi-Fontan) have had a completion FO without mortality or Fontan takedown. Risk factors for adverse outcomes were determined by multivariate analyses.There were no early but six late deaths. The follow-up period ranged from 1 year to 25 years (mean 10.0 +/- 6.5). The actuarial survival was 93% at 1 year, 87% at 10, 15, and 25 years. Multivariate analysis identified preoperative left or common atrioventricular valve insufficiency (P = .001) and decreased left ventricular ejection fraction (50%) (P = .02) as risk factors for late death and/or morbidity. The only risk factor for a late adverse outcome and poor functional status was increased preoperative pulmonary vascular resistance (4.0 Woods units/m(2)) (P = .005).FO provides good early- and mid-term results in the treatment of complex forms of DORV. The increased preoperative pulmonary vascular resistance was a significant negative risk factor for adverse outcome in this patient population.

Heparin-induced thrombocytopenia (HIT) is a life-threatening complication of heparin therapy. Anticoagulation in pediatric patients with HIT is challenging as there are no approved heparin substitutes. We report the use of the anticoagulant argatroban for cardiac surgery with cardiopulmonary bypass in an infant with double outlet right ventricle and a history of HIT.

Background. The hemodynamic profile and surgical approach to a double-chambered right ventricle (RV) are influenced by the position of a ventricular septal defect (VSD) in relation to the obstructing muscle bundle. This relationship, however, has not been systematically evaluated, with available literature yielding conflicting results. The objective of this study was to describe this relationship in a series of consecutive patients. Design: This is a retrospective study of patients with double-chambered RV at a single institution between 1999 and 2005. The location of the VSD in relation to the obstructing muscle bundle was established by a review of echocardiograms, cardiac catheterizations, and surgical notes. Results. Twenty-seven patients were identified, and 4 excluded because of inadequate data. There was no disagreement among the methods to determine the relationship between the VSD and the muscle bundle(s). The median age was 65 months (8–204 months). An associated VSD was seen in 21 patients (91%); 18 perimembranous (86%), 2 muscular (9%), and 1 supracristal (5%). The VSD was proximal to the obstructing muscle bundle in 13 (62%), and distal to the bundle in 9 (38%); the supracristal defect was distal, the 2 muscular were proximal, and 11 (61%) of the perimembranous defect were proximal to the muscle bundle. Conclusion. The position of the VSD in relation to the anomalous muscle bundle in a double-chambered RV is heterogeneous. The majority of defects communicate with the high-pressure chamber proximal to the muscle bundle(s).