Aortopulmonary window with pumonary atresia with ventricular septal defect with D-transposition of great arteries: extremely rare anomaly.

Aortopulmonary window (APW) is rare a congenital heart disease accounting for 0.1%-0.2% of all congenital heart defects. The 35% of the APW has been associated with wide variety of other structural heart diseases such as ventricular septal defect, persistent ductus arteriosus, arch anomalies and coronary artery anomalies. To the best of our knowledge, only six cases of APW with pulmonary atresia with ventricular septal defect has been described in the literature. It resembles the type 1 truncus arteriosus, and differentiation from this condition is important prior to surgical correction. We present a case of 14-year-old girl child; she was diagnosed with APW with pulmonary atresia with ventricular septal defect and D transposition of great arteries with the help of echocardiography, cardiac catheterisation and cardiac CT.

Aortic atresia with aortopulmonary window simulating common arterial trunk.

We describe the findings in a neonate with aortic atresia, interruption of the aortic arch between the carotid arteries, and aortopulmonary window. This unusual association changes the anticipated physiology, and helps in the survival. We emphasize the fact that this combination simulates common arterial trunk, both clinically and on echocardiography.

Sildenafil therapy in complex pulmonary atresia with pulmonary arterial hypertension.

BACKGROUND: Complex pulmonary atresia (CPA) i.e. pulmonary atresia with ventricular septal defect and major aorto-pulmonary collaterals (MAPCAs) or Tetralogy of Fallot with MAPCAs frequently have a clinical course complicated by development of pulmonary arterial (PA) hypertension. METHODS: A cross-sectional retrospective review of patients >16 years with CPA or Tetralogy of Fallot with MAPCAs and PA hypertension treated with sildenafil was conducted. Case notes were reviewed for baseline and follow-up (after sildenafil) characteristics. RESULTS: Five patients, 4 female, median age 28 (range 18 to 47) years, were identified. All patients experienced symptomatic improvement: 2 of 4 wheelchair bound patients responded dramatically and walked 345 and 157 m respectively in 6 min following sildenafil therapy. One of the 4 with marked PA arborization abnormalities and severe ventricular dysfunction had initial symptomatic improvement. Another patient improved from walking less 100 m to climbing 2 flights of stairs. Arterial saturations improved in 2 cases from 70 and 60% to 87 and 84% respectively, whilst arterial saturations remained static in 1 case despite embolization of a classical Blalock-Taussig shunt. One patient with PA arborization/diminished PA bed was unable to tolerate sildenafil. CONCLUSIONS: Sildenafil is well tolerated and leads to symptomatic improvement and better saturations in the majority of patients with CPA with PA hypertension when used in isolation or as an adjunct to percutaneous PA angioplasty.

Circulatory effects of sodium nitroprusside in the conscious lamb with an aortopulmonary left to right shunt.

We studied the effect on the circulation of reducing peripheral vascular resistance by infusing sodium nitroprusside into lambs of three different age groups (subgroup A, 11-26 days, subgroup B, 32-52 days, and subgroup C, 61-88 days of age) with and without an aortopulmonary left to right shunt. Infusion of 10 micrograms/kg/min nitroprusside over 2 h decreased aortic and left atrial pressures markedly and increased heart rate, whereas systemic, pulmonary, and left to right shunt blood flows hardly changed. Within 30 min after the onset of infusion, the hemodynamic variables stabilized. Aortic and left atrial pressures were still below control levels at that time. The different flows remained the same and heart rate, after an initial fall, increased again. The pattern of hemodynamic changes was not influenced by age or the presence of an aortopulmonary left to right shunt. Based on this study, we do not advocate sodium nitroprusside administration in case of a left to right shunt with normal arterial pressure and systemic blood flow.