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Objective: Absent pulmonary valve syndrome (APV) is a rare condition usually associated with tetralogy of Fallot (TOF). Some infants develop respiratory failure from bronchial compression and the long-term neurodevelopmental outcome is unknown. We aimed to investigate the outcomes of APV and the need for long-term ventilation (LTV).Design, Patients and Setting: Retrospective single-centre review of patients diagnosed with APV between 2007 and 2017.Outcome Measures: Survival, neurological disability and postoperative LTV (≥3 months of non-invasive or invasive respiratory support).Results: Thirty patients were identified, 22 (73%) of whom were prenatally diagnosed. Pregnancy was discontinued in one patient, while in utero death occurred in three. One was lost to follow-up. Of the remaining 25 liveborn, 21 had the classic TOF/APV. One baby died immediately after birth, while two patients had palliative care due to severe airway compression and inability to wean ventilation support. Surgical repair was performed in 21 of the 25 (84%) liveborn, with one awaiting surgery. Of those undergoing surgery, two patients died: one during surgery and the other due to severe airway malacia 5 months postsurgery. In the surgical group survival from birth at 1 and 5 years was 89% (95% CI 75% to 100%). Six (30%) patients required LTV postoperatively; all had surgery within the first 6 months of life. Learning and/or other physical difficulties were evident in 63%.Conclusions: Majority of patients with APV are diagnosed antenatally. A third of those operated required LTV and over half had learning and/or other physical difficulties. Prospective studies are needed to identify prenatal factors that predict postnatal outcomes so parents can be counselled appropriately. [ABSTRACT FROM AUTHOR]

Background: This study is a single-center experience with surgical repair of anomalous origin of left coronary artery from pulmonary artery (ALCAPA) with focus on the management of associated mitral regurgitation (MR). Methods: We performed a retrospective analysis of cases presenting to a quaternary referral center between November 1990 and October 2011. Results: In all, 25 patients (18 female) presented with a diagnosis of ALCAPA at a median age of 5 months (range, 1.5 to 102). Twenty-one patients (84%) had moderate to severe impairment of left ventricular function with median fractional shortening of 14% (range, 2% to 33%), and 19 patients (76%) had moderate to severe MR. Surgery was performed with direct coronary reimplantation in 16 patients (64%) and intrapulmonary tunnel (Takeuchi repair) in 9 (36%). Four patients had mitral valve repair at time of surgery, all for structural anomalies. Functional MR with a structurally normal mitral valve was not repaired. The median duration of postoperative follow-up was 93 months (range, 9 to 240). There were no early or late deaths, and no patient required mechanical support. Four patients (16%) required surgical or catheter reintervention. At last follow-up, 24 of 25 patients were asymptomatic; the left ventricular function was normal in 22 patients. Moderate MR was present in 4 patients. There was significant improvement in left ventricular function and MR (p < 0.01) during follow-up. Conclusions: Surgical repair of ALCAPA has good long-term results with low mortality and reintervention rates. The majority of MR is functional and will improve with reperfusion, but structural mitral valve abnormalities should be repaired at the time of surgery. [Copyright &y& Elsevier]

Coronary artery fistulae (CAF) are rare forms of congenital heart disease with an incidence of one in 50 000 live births. The authors present the case of an asymptomatic neonate with a precordial murmur. Pre and postductal saturations, blood pressure and ECG were normal. Echocardiography revealed a large right coronary artery fistula to the right ventricle (4.5 mm). At 11 months, transcatheter occlusion of the fistula with a vascular plug was performed. A year on, the child was thriving, ECG and echocardiogram remained normal. CAF complications and symptoms (including aneurysm, myocardial ischaemia, angina, heart failure and dyspnoea) are commoner in older patients, so traditionally we intervene early. With increasing case reports of spontaneous closure of even large and symptomatic fistulae, management of especially asymptomatic children is unclear. Long-term complications of intervention also remain largely unknown. As such more information is required on the conditions natural history to better manage patients and counsel parents. [ABSTRACT FROM AUTHOR]

The article presents case studies related to antenatally diagnosed aortico-left ventricular tunnel (ALVT). It states that the case of 20 weeks old antenatal, indicates the presence of perimembranous ventricular septal defect (VSD) having large aorta and dilated left ventricle. It mentions the case of a 22 weeks old antenatal with postoperative complications. It also discusses the neonate who was well at birth, in which the closure of the tunnel was performed using a bovine patch.

A 6-month-old boy with congenitally corrected transposition of the great arteries, ventricular septal defect, and mild pulmonary valve stenosis presented with acute obstructive respiratory life-threatening events. Airways obstruction was caused by aneurysmal dilation of the branch pulmonary arteries compressing the carina and bronchi. The child was ventilator dependent. A double switch operation with Lecompte maneuver was performed, bringing the pulmonary arteries forward and relieving the airway compression. The child made a complete recovery and remains well 9 months later with no clinical or radiologic signs of airway compression. [Copyright &y& Elsevier]