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Asymptomatic cardiac murmurs are a common finding in paediatric patients with some estimates suggesting that 90% of children have a murmur detected at some stage. Most are benign ‘innocent’ noises or reflect minor structural heart disease of no haemodynamic significance. The degree of parental anxiety invoked by the detection of a murmur in their child is considerable and although in virtually all cases their concerns of significant underlying cardiac disease are unjustified, providing adequate reassurance is a challenge for the health professionals involved. The aim of this article is to guide practitioners in determining which babies or children need urgent referral for a specialist opinion and to provide management suggestions for when a murmur is noted coincidentally during a routine examination.

ObjectiveWe sought to evaluate pulmonary artery banding in infants with complete atrioventricular septal defects.MethodsFrom 2000 to 2009, 20 infants with complete atrioventricular septal defects underwent pulmonary artery banding because of unsuitable anatomy (unbalanced ventricles, associated lesions, or both) or clinical condition (infection, chronic lung disease, or noncardiac malformation). Patients were divided into 2 groups: the conventional PAB group (n = 13 [65%]; mean age, 74 ± 56 days [range, 6–187 days]; mean weight, 3.3 ± 1.1 kg [range, 2.1–5.8 kg]) and the FloWatch-PAB group (n = 7 [35%]; mean age, 111 ± 40 days [range, 81–187 days]; mean weight, 4.3 ± 1.2 kg [range, 3.2–6.1 kg]). There was no statistical difference in age or weight. Preoperative mechanical ventilation was required in 3 (23%) of 13 infants in the conventional PAB group and 5 (71%) of 7 infants in the FloWatch-PAB group (P

Objective The Blalock–Taussig (BT) shunt is an excellent palliative procedure for cyanotic congenital heart defects. We reviewed two techniques of performing the BT shunt, median sternotomy and thoracotomy, in relation to morbidity and mortality. Methods Forty-five modified BT shunts in 41 patients, mean age 93 days (1–1045 days), were performed between January 2002 and October 2004. Twenty-four (53.3%) shunts in 21 (51.2%) patients were performed through thoracotomy and 21 (46.7%) shunts in 20 (48.8%) patients through median sternotomy. One surgeon preferred thoracotomy and the other sternotomy approach irrespective of age/weight or elective/emergency. Thirty-eight (84.4%) cases underwent elective operation and 7 (15.6%) cases were operated as emergencies. In both groups the most frequent diagnosis was complex Tetralogy of Fallot. Results Postoperative oxygen saturation was same in both groups and there were no significant complications in either group. Patients undergoing BT shunt via median sternotomy approach had longer duration of ventilation (mean 183h vs. 53h, P P P P Conclusions Median sternotomy approach to performing BT shunt seems to carry a higher morbidity than thoracotomy. We recommend a large case series study and longer follow up.

Surgical waiting lists are of high importance in countries, where the national health system is unable to deliver surgical services at a rate that would allow patients to avoid unnecessary periods of waiting. Prioritization of these lists, however, is frequently arbitrary and inconsistent.The objective of our research was to analyze the medical decision-making process when prioritizing patients with congenital cardiac malformations for cardiac surgical procedures, identifying an appropriate representation of knowledge, and transferring this knowledge onto the design and implementation of an expert system (“PrioHeart”).The medical decision-making process was stratified into three stages. The first was to analyze the details of the procedure and patient to define important impact factors on clinical priority, such as the risk of adverse events. The second step was to evaluate these impact factors to define an appropriate “timing category” within which a procedure should be performed. The third, and final, step was to re-evaluate the characteristics of individual patients to differentiate between those in the same timing category.We implemented this decision-making process using a rule-based production system with support for fuzzy sets, using the FuzzyCLIPS inference engine and expert system shell as a suitable development environment for the knowledge base.The “PrioHeart” expert system was developed to give paediatric cardiologists a tool to allow and facilitate the prioritization of patients on the cardiosurgical waiting list. Evaluation of “PrioHeart” on limited sets of patients documented appropriate results of prioritization, with a significant correlation between the prioritization made using “PrioHeart” and those results obtained by the individual consultant specialist.We conclude that our study has demonstrated the feasibility of using an expert system approach with a fuzzy, rule-based production system to implement the prioritization of cardiac surgical patients. The approach may potentially be transferable to other medical subspecialities.

SummaryWe report two cases with persistence of the hepatic venous complex, underdevelopment of the inferior caval vein and azygos continuation to the superior caval vein. We suggest that the hepatic venous complex is a true congenital malformation rather than the result of passive enlargement of venous channels subsequent to high venous pressures. Preoperative diagnosis is important in patients with complex congenital heart disease undergoing palliative venous redirection procedures if a postoperative right-to-left shunt and cyanosis are to be avoided.

This study sought to assess pregnant diabetic women for the presence of fetal cardiac anomalies and to determine whether better diabetic control was associated with a reduced risk to the fetus. Between 1988 and 1995, pregnant type I and II diabetic women routinely underwent fetal echocardiography. Hemoglobin A1c values were used as an indicator of maternal diabetic control and any relation between congenital heart disease in the fetus and maternal hemoglobin A1c levels was sought. Cardiac defects were identified in 7 of 328 pregnancies assessed, for an incidence of congenital heart disease of 2.1% (95% confidence interval: 0.6-3.6%). A review of the postnatal cardiac database did not reveal any undetected major malformations. The mean hemoglobin A1c level was 7.6% +/- 2.0% obtained at a mean gestational age of 12 +/- 7 weeks. Hemoglobin A1c levels of mothers carrying a fetus with congenital heart disease did not significantly differ from those with a normal fetus: 8.1% +/- 3.4% versus 7.6% +/- 1.9% (p = 0.48). Mothers with an affected fetus demonstrated a wide range of HbA1c levels (4.1 to 13.7%). Thus, the incidence of significant fetal cardiac abnormalities is low and not significantly related to maternal diabetic control.

Introduction: A multicenter prospective study was conducted to evaluate a new strategy for multiple Ventricular Septal Defects (VSDs). Materials and Methods: From 2004 to 2012 17 consecutive children (3 premature, 14 infants), mean age 3.2 months (9 days–9 months), mean body weight 4.2 kg (3.1–6.1 kg), with multiple VSDs underwent Pulmonary Artery Banding (PAB) with an adjustable FloWatch-PAB®. Associated cardiac anomalies included patent ductus arteriosus (1), aortic coarctation (2), hypoplastic aortic arch (2), and left isomerism (3). Five patients (5/17 = 29.4%) required pre-operative mechanical ventilation, with a mean duration of 64 days (7–240 days) Results: There were no early or late deaths during a mean follow-up of 48 months (7–98 months), with either FloWatch removal or last observation as end-points. FloWatch-PAB® adjustments were required in all patients: a mean of 4.8 times/patient (2–9) to tighten the PAB, and a mean of 1.1 times/patient (0–3) to release the PAB with the patient’s growth. After a mean interval of 29 months (8–69 months) 10/17 (59%) patients underwent re-operation: 7/10 PAB removal, with closure of a remaining unrestrictive VSD in 6 (peri-membranous in 3 patients, mid-muscular in 2, and inlet in 1) and Damus–Kaye–Stansel, bi-directional Glenn, and atrial septectomy in 1; 3/9 patients required only PAB removal. All muscular multiple VSDs had closed in all 10 patients. PA reconstruction was required in 1/10 patient. In 5/7 of the remaining patients with the PAB still in situ, all muscular VSDs had already closed. The only 2 patients with persistent muscular multiple VSDs are the 2 patients with the shortest follow-up. Conclusion: This reproducible new strategy with an adjustable PAB simplifies the management of infants with multiple VSDs and provides the following advantages: (a) good results (0% mortality), delayed surgery with a high incidence (15/17 = 88%) of spontaneous closure of multiple muscular VSDs, and facilitated closure of residual unrestrictive VSD (peri-membranous, mid-muscular, or inlet) at an older age and higher body weight; PAB with FloWatch-PAB® and its subsequent removal can potentially be the only procedure required for Swiss cheese multiple VSDs without an associated peri-membranous unrestrictive VSD.

Pulmonary atresia with intact septum is itself a rare congenital abnormality, albeit known to be associated with other cardiac and non-cardiac anomalies. The combination of right- and left-sided obstructive lesions, however, is extremely rare. We describe a patient having pulmonary atresia with intact septum associated with aortic coarctation, which to the best of our knowledge has been previously described on but one occasion.

The Blalock-Taussig (BT) shunt is an excellent palliative procedure for cyanotic congenital heart defects. We reviewed two techniques of performing the BT shunt, median sternotomy and thoracotomy, in relation to morbidity and mortality.Forty-five modified BT shunts in 41 patients, mean age 93 days (1-1045 days), were performed between January 2002 and October 2004. Twenty-four (53.3%) shunts in 21 (51.2%) patients were performed through thoracotomy and 21 (46.7%) shunts in 20 (48.8%) patients through median sternotomy. One surgeon preferred thoracotomy and the other sternotomy approach irrespective of age/weight or elective/emergency. Thirty-eight (84.4%) cases underwent elective operation and 7 (15.6%) cases were operated as emergencies. In both groups the most frequent diagnosis was complex Tetralogy of Fallot.Postoperative oxygen saturation was same in both groups and there were no significant complications in either group. Patients undergoing BT shunt via median sternotomy approach had longer duration of ventilation (mean 183 h vs. 53 h, P0.001) and inotropic requirements (33.3% vs. 4.2%, P0.05) leading to longer intensive care unit stay (mean 9.14 days vs. 3.3 days, P0.05) and hospital stay (mean 14.59 days vs. 5 days P0.005).Median sternotomy approach to performing BT shunt seems to carry a higher morbidity than thoracotomy. We recommend a large case series study and longer follow up.

Background Conflicting results have been obtained for the same operation for repair of coarctation of the aorta by different institutes. The purpose of this study was to assess the results of subclavian flap aortoplasty (SFA) alone, performed on 399 patients in a single institute between 1966 and 1995. Methods Data were collected retrospectively from the congenital cardiac surgical database at the institute. Results The median age at operation was 22 days (3 days–49 months). One hundred thirty-four patients had isolated coarctation while 265 children had complex coarctation. Maximum follow-up was 24 years (median, 14 years). Overall mortality over the whole duration of follow-up was 24.8%. Mortality for isolated coarctation at first intervention was 7.4% (operative mortality, 2.6%) while it was 12.8% for complex coarctation. At second intervention the mortality for isolated coarctation was 5%. For the second, third, and fourth interventions the mortality for complex coarctation was 25%, 25%, and 27%, respectively. The survival for isolated coarctation at 1, 5, 10, and 20 years was 94%, 93.2%, 92.4%, and 88.4%, respectively, while it was 74.6%, 66.3%, 63%, and 61.4%, respectively, for complex coarctation. Of the total patients, 15.3% had interventions for recoarctation. The incidence of recoarctation was 13.6% on those patients operated on in the first month of life, while it was 3.6% in older children. A percentage of 3.3% of patients continue to be hypertensive and require medication. There was a significant difference between the systolic blood pressure and anthropometric measurements between the arms. Despite this none of the patients complained of effect on lifestyle. Conclusions Despite improved early results the long-term mortality for coarctation remains high. Mortality is higher for complex coarctation as compared with isolated procedures. The incidence of recoarctation after SFA at long term is acceptable and is higher in patients operated on in the first month of life. The overall incidence of hypertension is quite low. Patients remained normotensive when operated upon at the age of 0.9 months. The SFA, no doubt, effects the limb development; however it does not cause limitation in the lifestyle.

SummaryWe report an unusual case with discordant atrioventricular and ventriculoarterial connections. In addition to the commonly associated intracardiac anomalies ofventricular septal defect and subpulmonary tissue tags, a supravalvar ring was present above the left-sided, morphologically tricuspid valve. This case highlights the value of color flow Doppler echocardiography in making the diagnosis, particularly as the supravalvar ring was seen poorly angiographically.

The objectives of this study are to assess the incidence of congenital complete heart block (CCHB) in pregnant women who are anti-Ro and/or La positive. Between January, 1988 and July 1997, 118 pregnancies in 105 women were assessed by fetal echo at 18, 24, and 32 weeks' gestation. Of these 105, 96 had no history of a previous fetus with CCHB; 11(12 pregnancies) a history of a pregnancy with CCHB; and 4 a previous child with cutaneous neonatal lupus erythematosus (CNLE). The 102 pregnancies in 96 women with anti-Ro and/or anti-La antibodies and no history of a previous child with NLB, resulted in 100 live-births with no CCHB. There was 1 dilated cardiomyopathy at 6 months, 1 child with CCHB, and 1 with sclerosis of the endocardium. No case of CCHB was observed in 102 pregnancies without a previous history of CCHB. These results suggest that the risks of CCHB, without a prior history are low.

OBJECTIVES—To determine the accuracy of prenatal diagnosis of pulmonary atresia and intact ventricular septum (PAIVS), and pulmonary stenosis, including prenatal detection of ventriculocoronary connections, to evaluate heart size during the prenatal period, and to evaluate the outcome. DESIGN AND PATIENTS—Medical records of 20 cases with prenatally diagnosed PAIVS and pulmonary stenosis were reviewed retrospectively. Prenatal and postnatal echocardiography were also reviewed and dimensions of the ventricles and vessels were measured retrespectively. RESULTS—Of 20 prenatal diagnoses (15 PAIVS and five pulmonary stenosis), 16 were confirmed as correct. One critical pulmonary stenosis case had been diagnosed as PAIVS prenatally; three had no confirmation. Eight pregnancies were terminated, three had no active treatment, and nine were treated; all survived. Of 13 assessed with ventriculocoronary connections prenatally, seven were diagnosed correctly (four with, three without ventriculocoronary connections), but one was falsely positive; five had no confirmation. The more prominent hypoplasia of the main pulmonary artery and the tricuspid valve annulus, and the sigmoid shape of the ductus arteriosus, seemed to be associated with the presence of ventriculocoronary connections. CONCLUSIONS—Current prenatal echocardiography can accurately diagnose right ventricular outflow tract obstruction and ventriculocoronary connections. Prenatal detection of this constellation of abnormalities aids in family counselling and decisions on postnatal management. Keywords: congenital heart disease; pulmonary atresia; ultrasonography; prenatal diagnosis

Hypocalcemia secondary to nutritional rickets is a rare cause of dilated cardiomyopathy. It is also not a recognized cause of dicarboxylic aciduria. We report the first case of adipic aciduria, presenting with dilated cardiomyopathy, secondary to hypocalcemia.

In a randomized controlled trial preterm babies received 20 mg vitamin E/kg im soon after birth (day 0) and at 24 and 48 h. The mean +/- SD plasma vitamin E level on day 0 was identical in supplemented and control groups (9.98 +/- 4.88 mumol/L) and rose progressively only in supplemented babies reaching a peak of 69.2 +/- 21.36 mumol/L at 72 h. Supplemented babies had a lower incidence of intraventricular hemorrhage (IVH) diagnosed by ultrasound (9/102, 8.8%) than the control group (37/108, 34.3%; 95% CI for difference in incidence, 15-36%). In a subsequent uncontrolled study using the same vitamin E preparation, a single dose of 20 mg/kg was given soon after birth to preterm babies. The peak mean +/- SD plasma level was 32.97 +/- 13.47 mumol/L at 48 h. The incidence of IVH (16/121, 13.2%) was lower than in historical control from the randomized trial (95% CI for difference; 10.2-31.8%) in spite of the single-dose group having more clinical risk factors for IVH. We conclude that vitamin E protects against IVH in preterm babies.

Background: The Blalock-Taussig shunt is considered a low-risk management option for palliation in tetralogy of Fallot, but the morbidity associated with a Blalock-Taussig shunt can have a significant impact on patient care. We reviewed the outcome for this operation in the current era. Methods: Between 1990 and 1994, 65 children with tetralogy of Fallot received a modified Blalock-Taussig shunt. Sixty patients who had follow-up angiography were assessed for clinical outcomes and shunt-related morbidity and mortality. From the same study period, 68 of 247 pediatric patients who underwent angiography and tetralogy repair, but did not receive palliation, were randomly selected to comprise a comparison group. Results: Palliation was more likely in the presence of a complicated tetralogy malformation or if there was an associated medical condition. Median age at palliation was 58 days (range: 1 to 535 days). Ninety-five percent of shunts were right-sided. Self-limited morbidity complicated 11% of shunt operations. Significantly smaller distal right pulmonary arteries were observed in the palliated group before total repair compared with findings in the group without palliation and 33% of patients who underwent palliation had angiographic evidence of pulmonary artery distortion. Shunt stenosis was common and correlated with younger age at palliation. Shunt occlusion resulted in one death. Excluding noncardiac causes of death, overall survival was 90% in the palliated group versus 97% in the nonpalliated group ( p = 0.09). Conclusions: Pulmonary artery hypoplasia and angiographic evidence of pulmonary artery distortion are common after initial palliation by a modified Blalock-Taussig shunt. Neonatal palliation was associated with significantly smaller pulmonary arteries before repair, which necessitated additional interventions(J Thorac Cardiovasc Surg 1997;114:25-30)