S.S. Kothari

Atrioventricular septal defect with cor triatriatum: Case report and review of the literature

Abstract. Atrioventricular septal defect with cor triatriatum is a rare combination, with only nine cases reported so far. Cor triatriatum, a potentially correctable cause of pulmonary arterial hypertension in atrioventricular septal defect patients, was missed in a few earlier case reports, leading to death and subsequent diagnosis at autopsy. We report the case of 3-year-old female child with this combination (partial atrioventricular septal defect with cor triatriatum) wherein the final diagnosis was made at cardiac catheterization. Successful surgical correction was performed.

Life-threatening arterial haemorrhage: Results of treatment by transcatheter embolization using home-made steel coils

Major arterial haemorrhage can be fatal. Its management by percutaneous embolization using commercially available embolizing agents is well established. We describe the use of home-made steel coils in the treatment of five patients. These coils were designed during the procedure in order to conform to the dimension of the bleeding artery. The group included two patients with life-threatening haemoptysis after surgical correction for tetralogy of Fallot; one patient each with exsanguinating haemorrhage from a branch of the left profunda femoral artery following violent trauma; massive haematuria due to an inoperable hypernephroma; and haemoptysis due to post-tubercular bronchiectasis. The bleeding vessel was evaluated by selective angiography in each patient. After assessing its length and diameter, steel coils of desired dimensions were made by utilizing a conventional 0.032-in wide guidewire and these were released into the bleeding artery. Occlusion of the bleeding vessel was obtained in all patients, without complication. Fabrication of coils of desired length and diameter can be done within a few minutes during the procedure. These coils provide an effective method of treating life-threatening arterial haemorrhage in selected cases.

Congenital severe aortic stenosis with congestive heart failure in late childhood and adolescence : Effect on left ventricular function after balloon valvuloplasty

Left ventricular (LV) dysfunction with congestive heart failure (CHF) resulting from severe congenital aortic stenosis (AS) is a well‐described condition in infancy, but it is rarely found in older children and adolescents. Aortic valve surgery in such cases may be associated with higher rates of morbidity and mortality. Aortic valve balloon dilatation (AVBD) is a viable alternative, but its effect on LV function has not been evaluated. We describe follow‐up results of AVBD in 10 cases of severe congenital AS in older children and adolescents with CHF and LV dysfunction. The ages of these patients ranged from 5 to 18 yr (mean ± SD: 10.8 ± 4 yr), and nine were males. The follow‐up period after AVBD ranged from 3 mo to 7 yr (mean ± SD: 2.93 ± 2.1 yr). Success was achieved in all cases, with no immediate complications. After valvuloplasty, the peak‐to‐peak systolic gradient declined from 74.7 ± 30.8 to 33.9 ± 18.2 mm Hg (P < 0.0001). The cardiac index increased slightly but significantly, from 1.9 ± 0.27 to 2.2 ± 0.5 L/min/m2 (P < 0.015). Hemodynamic improvement was also confirmed by a significant decrease in mean pulmonary artery and pulmonary artery wedge pressures from 41.9 ± 9 to 32.6 ± 6.6 and from 25.5 ± 2.9 to 19.3 ± 3.4 mm Hg, respectively. The echocardiographically derived left ventricular ejection fraction (LVEF) improved from 21.6 ± 5.37% to 31 ± 6.5% within 24 hr after AVBD, and it further improved in all cases on follow‐up. Mean LVEF at last follow‐up was 59.4 ± 11.4%. The Doppler instantaneous peak systolic gradient (IPSG) increased from 37.3 ± 18.8 to 64.8 ± 30.7 mm Hg at late follow‐up. Significant aortic regurgitation (AR) developed in 20% of patients. The Doppler IPSG across the aortic valve was > 60 mm Hg in five cases on follow‐up. Two of these patients underwent another AVBD successfully 4 and 16 mo later, respectively. Aortic valve replacement was done in two patients, one for severe restenosis with mild AR 12 mo after AVBD and another for severe re‐restenosis with moderate AR 21 mo after a second AVBD. Severe congenital AS can be associated with LV dysfunction and CHF in late childhood and adolescence. AVBD results in good palliation with improvement in LV function on follow‐up. Cathet. Cardiovasc. Intervent. 51:168–172, 2000.

Persistent Truncus Arteriosus with Double Aortic Arch

We report a rare association of persistent truncus arteriosus with double aortic arch in a 34-day old neonate.

Fatal Salmonella typhimurium Infection of a Vascular Graft in an Infant

A case of Salmonella typhimurium endocarditis of a Blalock-Taussig shunt in an infant is described for its rarity. Wider appreciation of such infections is warranted.

Superior vena caval obstruction after complete resolution of cardiac tuberculoma

Superior vena cava obstruction (SVCO) is uncommon. It usually results from extrinsic compression/ invasion from mediastinal masses, with the majority of the lesions being malignant. Rarer causes include mediastinal lymphadenopathy, benign tumours, fibrosing mediastinitis, thyroid enlargement, aortic aneurysm, or placement of pacing leads and catheters. Cardiac involvement in tuberculosis most often manifests as tuberculous pericarditis. Involvement of the myocardium is reported in up to 0.3% of cases dying of tuberculosis. Association of SVCO with cardiac tuberculoma is extremely rare, with only one case reported in 1973. We describe the imaging findings in a patient who developed persistent SVCO after complete disappearance of a cardiac tuberculoma on anti-tuberculous therapy (ATT).

Percutaneous transvenous mitral commissurotomy in patients with severe mitral stenosis and acute rheumatic fever.

There is a concern that percutaneous transvenous mitral commissurotomy (PTMC) performed during acute rheumatic fever (ARF) may result in adverse outcomes. We performed a retrospective study at a tertiary care center. Ten patients (ages 8–12 years) with severe symptomatic mitral stenosis and ARF who underwent PTMC (ARF group) were compared with 10 controls with similar mitral valve area (MVA). The procedure was successful in all patients. The median MVA increased from 0.7 to 1.5 cm2 following PTMC in the ARF group and from 0.7 to 1.8 cm2 in the control group [p = not significant (NS)]. The median MVA was 1.2 cm2 in the study group compared to 1.6 cm2 in the control group (p = 0.02) over a mean follow-up of 17.5 ± 11.7 months. Echocardiographic restenosis occurred in 4 patients (40%) in the ARF group compared to 1 patient (10%) in the control group (p = NS). The improvement in the New York Heart Association functional class was maintained in 8 patients of the ARF group and 9 patients of the control group. PTMC can be performed in children with ARF without an increase in procedural complications. However, the restenosis rates appear to be higher.

Liver herniation into the pericardium: an unusual cause of massive pericardial effusion with intrapericardial mass in a neonate.

In neonates presenting with massive pericardial effusion and pericardial mass, liver herniation into the pericardium is a rare diagnosis. Echocardiogram and CT scan are useful investigations in the diagnosis of pericardial masses. Continuation of the mass with the liver with the same texture as the liver helps to make the diagnosis of intrapericardial herniation of liver. Correct diagnosis of this condition is important because the surgical approach needed for management of this condition is different from that used for other pericardial masses.

Obstructed Infracardiac Total Anomalous Pulmonary Venous Connection in an Adult

Adult survival in patients with uncorrected obstructed infracardiac total anomalous pulmonary venous drainage has not been reported. A 17-year-old man who presented to us with features of severe pulmonary arterial hypertension was diagnosed to have obstructed pulmonary venous drainage to the hepatic vein. Presence of a large ventricular septal defect in this patient may have contributed favorably to survival.

Single-Stage Repair of Interrupted Aortic Arch and Taussig–Bing Anomaly

We report a case of successful single-stage repair of interrupted aortic arch with Taussig–Bing anomaly in a 5-month-old infant.