Biomedical subjects
Publications and source records attributed to Shyam S Kothari.
A 45-day-old infant presented with the unusual and intriguing symptom of episodic crying and loss of consciousness. The infant was discovered to have a vascular compression of the trachea by the innominate artery, almost serendipitously. He was cured of his symptoms by anterior suspension of the innominate artery.
BACKGROUND: The aim of this study was to ascertain the clinical course and prognosis of dilated cardiomyopathy in Indian children. METHODS AND RESULTS: The records of 82 children with dilated cardiomyopathy (50 males), less than 12 years of age (mean age 2.9+/-3.07 years), were retrospectively reviewed. Clinical variables, laboratory parameters, and serial echocardiograms were analyzed. On a mean follow-up of 25.09 months (range 15 days-118 months), 9 out of 78 patients died (11.5%) (CI: 4.5%-18.5%). Mortality was 25% (6/24) in infants but the actuarial survival was 87% at 5 years in those diagnosed beyond infancy. Serial echocardiograms of 66 patients (80%) were available. Of these, 39 patients (59%) (CI: 47%-70%) improved, 12 (18%) (CI: 9%-27%) deteriorated or died, and 15 (23%) (CI: 13%-33%) remained unchanged during the follow-up. Among the prognostic variables, only age less than 1 year, higher cardiothoracic ratio, and a higher ratio of left ventricular diastolic dimension/posterior wall thickness was associated with a poor outcome on univariate analysis. CONCLUSIONS: Dilated cardiomyopathy in children pursues a heterogeneous course with a high mortality in infants. A large number of children diagnosed beyond infancy improve or recover. Further characterization of prognostic variables is warranted.
Surgical or interventional perforation of atretic pulmonary valve as initial palliative procedure has been advocated in patients with pulmonary atresia with an intact ventricular septum. We report two infants with pulmonary atresia and intact ventricular septum, who were treated by radiofrequency perforation and balloon dilatation.
BACKGROUND: There is no consensus about the most appropriate limits of pulmonary artery pressure and vascular resistance in case of patients undergoing univentricular or one and one-half ventricular repair. This study was conducted to analyze the mortality and morbidity of a heterogenous group of patients with a functionally univentricular heart and pulmonary artery hypertension, undergoing pulmonary artery banding followed by univentricular-type repairs. METHODS AND RESULTS: Out of 254 patients undergoing pulmonary artery banding for a functionally univentricular heart with increased pulmonary blood flow, 148 patients underwent definitive second stage surgery. Post-band hemodynamic evaluation revealed persistently high pulmonary artery pressure (> 18 mmHg), and pulmonary vascular resistance (>2.0 Woods units/m2) in 78.3% patients. Sixteen patients with moderate right ventricular hypoplasia were given a one and one-half ventricle repair (Group I), 82 patients a bidirectional Glenn connection (Group II), and 50 patients a fenestrated total cavopulmonary connection (Group III). The overall mortality following second stage surgery for the high pulmonary artery pressure group (n=116) was 30.17%, while none of the low pulmonary artery pressure group died (p=0.0009). Pulmonary hypertensive crises and/or systemic desaturation were the main causes of death at second stage repair. All mortality occurred in patients with mean pulmonary artery pressure > 18 mmHg and pulmonary vascular resistance > 3.5 Woods units/m2. Survivors from this group had persistent morbidity in the form of superior vena caval syndrome and suboptimal oxygen saturation (70-75%). CONCLUSIONS: It is advisable not to proceed with definitive second stage repair if post-pulmonary artery banding mean pulmonary artery pressure is over 25 mmHg and pulmonary vascular resistance exceeds 4.0 Woods units/m2. These patients may possibly be deemed to have undergone definitive palliation during their pulmonary artery banding.
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A 10-year-old boy with tetralogy of Fallot and congestive heart failure underwent a right-sided modified Blalock-Taussig anastomosis because of severe biventricular dysfunction and repeated hypercyanotic spells. Postoperatively, there was improvement in systemic oxygen saturation and myocardial function. We postulate that congestive heart failure occurred because of severe myocardial hypoxia and its elimination resulted in markedly improved cardiac performance.
A 32-year-old man with 17 mm secundum atrial septal defect showed hemodynamic deterioration during temporary balloon occlusion of the defect. The closure was not done. Further investigation led to the diagnosis of primary cardiac amyloidosis.