Pulmonary atresia with an intact ventricular septum is characterised by heterogeneity in right ventricle morphology and coronary anatomy. In some cases, the presence of ventriculocoronary connections may promote coronary artery stenosis or interruption, and aortic diastolic pressure may not be sufficient to drive coronary blood flow. This requires a correct evaluation (currently done by angiography) which depends on whether the patient can be offered decompression of the right ventricle. To date, there is no objective method to do so, so we designed a percutaneous, transitory technique with the purpose of occluding the transtricuspid anterograde flow. The manoeuverer was performed in a 25-day-old female with pulmonary atresia with intact ventricular septum, right ventricle at suprasystemic level, and selective coronarography was not conclusive, the anterior descendant with stenosis in its middle third and from this point, thinner with to-fro flow. Occlusion was performed with a balloon catheter. We re-evaluated the coronary flow and the normalised anterior descendant flow. We hope that with this new method, we can give a more accurate diagnosis and determine the cases in which the coronary circulation is truly not right ventricle dependent to offer a greater number of patients biventricular or 1.5 ventricular repairs and thereby improve their quality of life and survival, the ones that turn out to be right ventricular dependant; offer them an early reference for cardiac transplant or in case it is not available to consider univentricular palliation knowing that this probably would not reduce the risk of ischaemia and/or death over time.

Pulmonary atresia with intact ventricular septum is a complex cyanotic congenital heart lesion with the potential for myocardial ischaemia due to the presence of coronary artery anomalies. We present a case of a two-day-old baby with postnatal diagnosis of pulmonary atresia with intact ventricular septum in whom non-invasive ferumoxytol-enhanced 4D cardiac magnetic resonance angiography was used for the assessment of coronary artery anatomy.

We report the first successful Glenn procedure for pulmonary atresia with intact ventricular septum in the setting of D-transposition of the great arteries. This case was complicated by hypoplastic left ventricle and severe mitral regurgitation. Partial closure of the mitral valve at the time of Blalock-Taussig shunting resulted in improvement of heart failure.

Left ventricular outflow tract obstruction in patients with pulmonary atresia with intact ventricular septum has been rarely reported. Data are lacking on the impact and management of systemic ventricular outflow tract obstruction that developed following the Fontan procedure. We report a case of an 8-year-old male who developed left ventricular outflow tract obstruction 6 months after the Fontan procedure.

Coronary abnormalities are frequent in pulmonary atresia and intact ventricular septum, mainly in patients with a very diminutive right ventricle. They severely impact on early and late prognosis. We describe an 8-year-old girl who presented with myocardial ischaemia, late after uneventful Fontan completion. The importance of precise delineation of the coronary anatomy upon initial assessment and during follow-up is emphasised.

In this study, we describe an infant case of pulmonary atresia with intact ventricular septum associated with ventriculo-coronary arterial communication for which a modified Blalock–Taussig shunt operation was performed. He experienced repeated myocardial ischaemic attacks. Further examination revealed pulmonary sequestration in the right lower lobe. He therefore underwent a bidirectional Glenn operation and coil occlusion of the feeding arteries. His myocardial ischaemic attacks subsequently improved.

Neonates born with hypoplastic left heart syndrome now have a remarkably improved prognosis compared with the situation existing before the development of the Norwood sequence of operative procedures. Some of those born with hypoplastic right ventricles in the setting of pulmonary atresia with an intact ventricular septum, however, still have a relatively poor prognosis. In part this reflects the presence of fistulous communication between the cavity of the right hypoplastic right ventricle and the coronary arterial tree. Such fistulous communications are now increasingly recognised as being important in the setting of hypoplastic left heart syndrome. In this brief review, we describe the anatomy of the communications. Those found with hypoplastic right ventricles are seen most frequently when the cavity of the ventricle effectively represents only the inlet, this in turn reflecting mural overgrowth of the apical trabecular and outlet components during foetal development. This almost certainly reflects an earlier appearance of the pulmonary valvar lesion that promotes the cavitary hypoplasia. In those with hypoplastic left ventricles, the key feature differentiating those with fistulous communications is the presence of a patent mitral valve, since the left ventricle is typically no more than a virtual slit in postero-inferior ventricular wall in the setting of mitral valvar atresia or absence of the left atrioventricular connection.

Described is a rare association in a patient with the heart in the left chest, namely pulmonary atresia with intact ventricular septum, fistulous coronary arterial connections, a right-sided aortic arch and an aorto-pulmonary collateral artery feeding one lung. The pulmonary arteries were non-confluent, with the right lung supplied by the right arterial duct originating from the under surface of the right-sided aortic arch, and the left lung supplied through the aorto-pulmonary collateral artery arising from the descending aorta. The surgical management is different in the setting of non-confluent pulmonary arteries.

The management of pulmonary atresia with intact ventricular septum remains controversial. The plan for management at our institution is based on the echocardiographic identification of a well-developed infundibulum. Neonates with such a well-developed infundibulum are prepared for a biventricular repair, whereas those without an infundibulum are groomed for a Fontan operation. In all, 48 neonates with the lesion were admitted to our unit between 1980 and 1992. In 31 neonates with a well-formed infundibulum, the initial palliation consisted mainly of pulmonary valvotomy without cardiopulmonary bypass and construction of a polytetrafluoroethylene shunt from the left subclavian artery to the pulmonary trunk. In most of these patients, growth of the right ventricle could be promoted by establishing antegrade flow through the ventricle. In seven patients in this group, nonetheless, the right ventricle failed to grow satisfactorily. In this subgroup, additional intermediate procedures were performed on the apical component and infundibulum of the ventricle together with the tricuspid and pulmonary valves which could collectively be termed as “right ventricular overhaul.” Five of these patients have gone on to further successful biventricular repairs at a median interval of 24 months after the overhaul. There was no operative mortality in this group. Following the final biventricular repair, there have been no late deaths or reoperations over a total follow-up of 145.4 patient months (mean 22.2 months).

A patient with the rare combination of an unusual form of pulmonary atresia with intact ventricular septum and a Marfan-like disease is presented. She was extremely cyanosed in childhood but improved after construction of a left peripheral shunt at the age of nine years. She deteriorated gradually after the onset of atrial fibrillation at the age of 33, the subsequent course being complicated by a stroke and, finally, by intractable heart failure and death at the age of 39.

The authors describe the prenatal echocardiographic diagnosis of a rare case of pulmonary atresia with intact ventricular septum, right-sided aortic arch, and ventriculocoronary connection in a fetus at 21 weeks gestation. The diagnosis was confirmed at autopsy.

As far as we are aware, a bicuspid aortic valve has not previously been reported in the setting of tetralogy of Fallot and pulmonary atresia. We describe this association in a newborn who presented with a murmur and cyanosis. Echocardiography showed tetralogy of Fallot with pulmonary atresia, and also a moderately stenotic bicuspid aortic valve. The patient underwent open-heart surgery guided by transesophageal echocardiography. Postoperatively, there was only mild obstruction across both outflow tracts. We have also reviewed the pertinent data from our Jesse E. Edwards Registry of Cardiovascular Disease to establish the incidence of bicuspid aortic valve in the setting of obstruction of the right ventricular outflow tract, finding the association in no patients with pulmonary atresia and tetralogy of Fallot, in 0.7% of those with tetralogy of Fallot and pulmonary stenosis, but in 6.6% of those with pulmonary atresia and intact ventricular septum.