Sunil K. Kaushal

Coronary Artery Fistulas in Pulmonary Atresia and Ventricular Septal Defect

Pulmonary atresia with ventricular septal defect is an anomaly with highly variable anatomy. Rarely, a coronary artery-to-pulmonary artery fistula may contribute to pulmonary blood flow. Since 1996, we have treated 4 patients with coronary-pulmonary fistula associated with pulmonary atresia and ventricular septal defect. Two fistulas originated from the left coronary, one from the right coronary, and one from a right-sided solitary coronary system. All terminated in the main pulmonary artery, which was adequate in all cases. The fistulas were managed by direct internal closure. Total intracardiac repair was then accomplished in all patients at the same sitting. There was one death. In children with favorable anatomy, direct closure of the fistula from the pulmonary artery is adequate and allows single-stage intracardiac repair.

Surgical experience with total correction of tetralogy of Fallot in infancy

Fifty two patients less than one year old with tetralogy of Fallot underwent primary repair between January 1991 and December 1994. Age range was three to twelve months (mean 10.09 +/- 2.01 months) and body weight ranged from 4.5 to 9 kg (mean 8.38 +/- 2.79 kg). Transatrial-transpulmonary repair was performed in 36 patients and the classical transventricular approach was used in 16 patients. Six patients underwent emergency surgery for severe cyanosis and spells. Five patients had left pulmonary artery plasty for pulmonary artery bifurcation stenosis and two out of the five patients who had anomalous coronary arteries needed a right ventricle to pulmonary artery conduit. Mean post repair peak right ventricular/systemic pressure ratio was 0.74 +/- 0.18 in the transventricular group and 0.71 +/- 0.26 in the transatrial-transpulmonary group. There were three hospital deaths. Follow-up ranged from 3 to 46 months (mean 21.18 months). Forty patients underwent echocardiography and twenty patients underwent cardiac catheterisation six to eighteen months after surgery. Mean right ventricular outflow tract gradient on echocardiography was 20.35 +/- 10.12 and, at cardiac catheterisation, 17.51 +/- 13.49 mmHg with mean post repair peak right ventricle/left ventricle pressure ratio of 0.44 +/- 0.11. These were significantly less than the values obtained in the operating room. Only one patient had residual ventricular septal defect with left to right shunt of 1.6:1 at cardiac recatheterisation. There was one late death after reoperation for residual obstruction. Encouraging results with primary repair of tetralogy of Fallot in infancy prompt us to continue this policy in suitable cases.

Total anomalous systemic venous drainage to the left atrium: An entity reviewed and investigated

We present three unusual cases of total anomalous systemic venous drainage (TASVD) where all the systemic veins were abnormally connected to the atrium that drained into the left ventricle (LV). All three patients had features consistent with left atrial isomerism. Based on the available evidence and review of literature we propose that TASVD be included as a part of the syndrome of left atrial isomerism. A classification for TASVD is also proposed with surgical implications.

Inferior-type caval vein defect--echocardiographic and surgical description of a large series of patients.

AIM This study was carried out to define the anatomical criteria for the diagnosis of inferior-type caval vein defect and compare the echocardiographic findings with surgical findings. METHODS The records of 19 patients - 13 male and six female patients in the age group of 18 months to 27 years, who were diagnosed as inferior-type caval vein defect with or without anomalous drainage of right pulmonary vein(s) on echocardiography - were retrospectively reviewed and compared with surgical findings. RESULTS Surgical diagnosis of inferior-type caval vein defect was confirmed in 17 of the 19 patients. In two patients, the surgical diagnosis was that of a large fossa ovalis atrial septal defect - confluent defect and fossa ovalis atrial septal defect with deficient inferior rim in one patient each. Surgical diagnosis of anomalous drainage of pulmonary vein(s) was based on the course of the superior rim of the defect in relation to the pulmonary veins. Our echocardiographic impression of the pulmonary veins appearing in its normal position but showing abnormal drainage to right atrium was in agreement with the surgical notes. Discrepancy was found in the number of pulmonary veins draining anomalously. The discordance was related to overdiagnosis of anomalous drainage in all except one, that is, three out of four. In one, only the right lower pulmonary vein was diagnosed to be anomalous, whereas both right upper and lower pulmonary veins were found to be anomalous. CONCLUSIONS Echocardiography provides definite diagnosis of inferior-type caval vein defect. Inferior caval vein straddling and an intact fossa ovalis are prerequisites for diagnosis. Anomalous pulmonary venous drainage of the right pulmonary veins is very common in our series, although accurate diagnosis of the number of pulmonary veins was not possible in all cases. Multiple views on transthoracic echocardiography starting from the subxiphoid views delineate the morphology accurately. Transoesophageal echocardiography is required only in patients in whom the windows, especially the subxiphoid, are not adequate.

Spontaneous Resolution of Residual Mitral Regurgitation in Patient With ALCAPA on ECMO.

We describe a case of revascularization for anomalous left coronary artery from the pulmonary artery (ALCAPA) with severe left ventricular (LV) dysfunction and severe mitral regurgitation (MR). Extracorporeal membrane oxygenation (ECMO), later successfully converted to an indigenous left ventricular assist device (LVAD) functionally resulted in the spontaneous resolution of MR and satisfactory recovery of LV function.

Criss-cross heart with supero-inferior ventricles: successful surgical correction of associated defects

We present an unusual case of criss-cross heart with supero-inferior ventricles, double outlet of the right ventricle, restrictive ventricular septal defect, severe infundibular stenosis and “absent” pulmonary valve who underwent successful surgical repair. Complete preoperative diagnosis was established with cross-sectional echocardiography combined with colour Doppler and angiocardiography, which facilitated successful surgical correction.

MINIMALLY INVASIVE LIGATION OF CORONARY ARTERY FISTULA

Successful surgical occlusion of a left anterior descending coronary artery-to-right ventricular fistula through a minimally invasive route is described. The fistula was ligated epicardially through a small midline pericardial window created in the xiphisternum without myocardial dissection, cardiac arrest, or cardiopulmonary bypass. Intraoperative transesophageal echocardiography played a crucial role in locating the site of the fistula, thereby allowing a minimally invasive surgical approach and assessment of the adequacy of repair.

Surgical Experience with Doty's Repair for Supravalvular Aortic Stenosis

The surgical treatment of severe supravalvular aortic stenosis by conventional, asymmetrical, one point patch aortoplasty across the narrowed area has been associated with a substantial incidence of residual stenosis and reoperations. Almost symmetrical relief of such stenosis was achieved with Dotys extended aortoplasty in 5 patients in whom left ventricular aortic gradient was reduced from 60–170 nun Hg to 0–25 mm Hg. This technique of inserting an inverted V-shaped gusset across the stenosed segment into the right coronary sinus and non-coronary sinus, restores the aortic root geometry, provides more predictable relief of aortic obstruction and also improves aortic valve function. Associated procedures involved relief of coarctation of aorta and innominate artery stenosis in 1 case and repair of aortic valve and mitral valve in another case.