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Biomedical subjects

S S Kothari

Publications and source records attributed to S S Kothari.

At least 127 records · Page 7Linked to original sources
PubMedDate not supplied

Surgical considerations of univentricular heart with total anomalous pulmonary venous connection.

Out of the 600 patients undergoing univentricular repair during the last 11 years, 20 children had associated total anomalous pulmonary venous connection. The objective was to outline the clues to establish the diagnosis of this rare disease combination and the various surgical options available to manage the same. Bidirectional Glenn, bilateral bidirectional Glenn, total cavopulmonary connection and atriopulmonary connection were performed in combination with rechannelling of various types of total anomalous pulmonary venous connection in 20 children aged 6 months to 36 months (mean +/- SD 17.65 +/- 9.02 months). Diagnosis could be established pre-operatively in only 13 (65%) patients. Out of 6 early deaths (30%), 4 were directly attributable to missed diagnosis. No late deaths occurred over a follow-up period ranging from 1 month to 132 months. None of the surviving children required reoperation and all are in NYHA functional class I. Doppler echocardiography of the surviving children revealed unrestricted atrio/cavopulmonary anastomosis and pulmonary vein to atrium connection in all survivors. Our own experience, coupled with a review of the literature, indicates that a missed diagnosis increases the hospital mortality. Cross sectional 2D echocardiography is a superior method of detection of associated total anomalous pulmonary venous connection compared to angiocardiography. Exclusion of the diagnosis of anomalous pulmonary venous connection is imperative in all univentricular hearts pre-operatively and on operation table. Failure to recognise this disease combination results in formation of a closed systemic circuit after bidirectional Glenn or a modified Fontan of connection and is lethal as happened in our early experience. It is suggested that one-stage Fontan operation should be performed only if other criteria for Fontan procedure are satisfied.

Cardiac Surgical Procedures↗
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Cardiac arrhythmias in surgically repaired total anomalous pulmonary venous connection: a follow-up study.

Twenty-five patients with diagnosis of total anomalous pulmonary venous connection, who had undergone corrective surgery, were studied at variable time period after surgery with 24-hour ambulatory electrocardiographic monitoring (Holter) and echocardiography. The aim of this study was to record arrhythmias, if any, and to correlate occurrence of arrhythmia with adequacy of repair and other related variables. All the patients were clinically asymptomatic. Twenty-four hours ambulatory electrocardiographic monitoring of these patients showed the presence of significant arrhythmias in 21 of the 25 patients. These included supraventricular ectopics in 19 patients, ventricular ectopics in 8, atrioventricular block in 2, right bundle branch block and atrial fibrillation 1 each and atrial tachycardia in 2 patients. There was no correlation between development of arrhythmia and age at repair, type of connection, operative approach and adequacy of repair. The study indicates that cardiac arrhythmias can occur in otherwise asymptomatic patients after correction for total anomalous pulmonary venous connection. Thus, these patients require long-term follow-up, even if they are asymptomatic.

Adolescent↗
PubMedDate not supplied

Surgical considerations of extracardiac total cavopulmonary connection.

Extracardiac total cavopulmonary connection has been proposed as a rational alternative to the lateral intra-atrial tunnel for complex congenital cyanotic heart diseases undergoing univentricular repair. In five patients, aged 4 1/2 years to 27 years, an extracardiac lateral conduit was used for total cavopulmonary connection at our Institute. Extracardiac lateral conduits were constructed in all the five patients using polytetrafluoroethylene tube grafts. One child required reoperation because of graft thrombosis and an aortic homograft was used in him on the second occasion. Aortic cross clamp was completely avoided in all including reoperation. The results showed no early or late mortality. One child developed persistent hypoxemia and progressive hepatomegaly. Reoperation on 5th post-operative day revealed graft thrombosis with technically unrestricted anastomosis which was replaced by an aortic homograft. He also required embolectomy for inferior vena cava and hepatic veins thrombosis. Post-operative follow-up (range 3 months to 14 months) revealed all patients in functional class I and in normal sinus rhythm. Doppler echocardiography, magnetic resonance imaging and angiocardiography revealed unrestricted anastomoses and unobstructed pulmonary blood flow across the conduit. The technical advantages and haemodynamic benefits of this operation are encouraging. However, the lone incidence of graft thrombosis raises concern about the routine usage of synthetic grafts in extracardiac total pulmonary connection. Other inherent weakness appears to be the lack of growth potential of the synthetic tube.

Adolescent↗
PubMedDate not supplied

Does inhaled nitric oxide improve survival in operated congenital disease with severe pulmonary hypertension?

BACKGROUND: The present study aimed to assess the impact of inhaled nitric oxide on survival following correction of congenital heart defects with residual pulmonary arterial hypertension. METHODS AND RESULTS: Inhaled nitric oxide was utilized for the management of residual pulmonary hypertension in 24 children following surgical correction of their underlying heart defects. Their ages ranged from 15 days to 14 months (median 5 months). Pulmonary artery hypertension was diagnosed either by direct pulmonary artery pressure monitoring or by echocardiography. Inhaled nitric oxide was used electively in 22 patients when the ratio of the mean pulmonary arterial pressure and mean systemic arterial pressure exceeded 0.5. In the remaining 2 patients, nitric oxide was used only to manage a pulmonary hypertensive crisis. Inhaled nitric oxide was also used a second time in 2 patients who developed delayed pulmonary hypertensive crisis. Twenty-two patients showed an initial response to therapy and the pulmonary artery pressures dropped significantly. Of the patients on direct pulmonary artery pressure monitoring, a pulmonary artery to systemic artery pressure ratio below 0.3 on prolonged therapy was associated with a survival ratio of 4/6 (including 1 neurological death and one reoperation); that between 0.3 and 0.5 with a survival ratio of 3/4. Three out of four patients with sustained echocardiographic and clinical response also survived and were discharged from the hospital. All the patients who showed a lack of response to (n=2), tolerance to (n=1), or dependence on (n=6) the use of inhaled nitric oxide died. In addition, all 5 patients who had a pulmonary hypertensive crisis died, 3 in spite of successful resuscitation with nitric oxide. Thus, excluding one neurological death and one re-operation, only 9 (41%) out of 22 patients survived. CONCLUSIONS: Though inhaled nitric oxide is effective in lowering pulmonary pressure, it does not appear to improve the survival rate following repair of congenital heart disease in those with associated severe pulmonary hypertension. A randomized trial between the use and non-use of inhaled nitric oxide is warranted to determine its exact role in influencing survival in patients with residual pulmonary hypertension following surgical repair.

Administration, Inhalation↗
PubMedDate not supplied

Aneurysm of the vein of galen in neonates: report of four cases.

In neonates, aneurysm of the vein of Galen often masquerades as cyanotic congenital heart disease. We report 4 cases of neonates presenting with malformation of the vein of Galen at our insititution. An increased awareness of this entity seems warranted.

Cerebral Veins↗
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Total anomalous pulmonary venous connection: surgical experience in Indians.

BACKGROUND: We report a retrospective analysis of the demographic. morphological and clinical profiles of patients along with results of operative repair for total anomalous pulmonary venous connection. METHODS AND RESULTS: In the last 15 years, 248 patients (168 boys, 80 girls) underwent repair for total anomalous pulmonary venous connection. Their ages ranged from I day to 24 years (median 8 months) and 145 of them were < or = 1 year of age. The patients' weight ranged from 2 to 52 kg (median 5 kg). About 70% of patients (n = 174) were less than the 50th percentile of predicted weight for age and sex. The anomalous connection was supracardiac in 134 (54%), cardiac in 80 (32.2%), infracardiac in 9 (3.6%) and mixed in 25 (10.1%) patients. Fifty (20.2%) patients had obstructed drainage and 76 patients (30.2%) had moderate or severe pulmonary arterial hypertension. Forty-five patients (18.1%) had to be operated upon on an emergency basis. All the patients were operated upon using moderately hypothermic cardiopulmonary bypass. In 114 patients, circulatory arrest was used. There were 45 (19.1%) in-hospital deaths. The major causes of early death were pulmonary arterial hypertensive crisis in 19 (7.7%) and low cardiac output syndrome in 17 (6.9%) patients. Age < or = 1 year (odds ratio 2.16; 95% confidence interval: 1.22-3.82, p=0.008), severe pulmonary arterial hypertension (odds ratio 5.86; 95% confidence interval: 2-17, p=0.001), and need for emergency surgery (odds ratio 3.65; 95% confidence interval: 1.59-8.38, p=0.002) were independent risk factors for early death. Follow-up ranged from 1 to 180 months (median 48 months). There were 4 lake deaths. Actuarial survival at 12 years was 92.6% +/- 2.8%. CONCLUSIONS: In Indian circumstances, mortality continues to be high in infants with total anomalous pulmonary venous connection. Severe pulmonary arterial hypertension appears to be the most important predictor of operative mortality. Severe malnutrition, delayed diagnosis and late referrals possibly contribute to the high mortality.

Adolescent↗
PubMedDate not supplied

Graded balloon atrial septostomy in severe pulmonary hypertension.

BACKGROUND: The prognosis of patients with severe primary pulmonary hypertension is poor. The role of balloon atrial septostomy as a palliative procedure in these patients is not well defined. We retrospectively analyzed our data regarding the safety, clinical outcome and survival benefit of graded balloon atrial septostomy in patients with severe pulmonary hypertension. METHODS AND RESULTS: Eleven patients (7 males), aged 6 to 30 years (mean age 16.2+/-8.9 years), with severe pulmonary artery hypertension (mean pulmonary artery pressure of 76+/-16.9 mmHg) and refractory congestive heart failure and/or recurrent syncope underwent balloon atrial septostomy. Graded balloon dilatation under echocardiographic guidance and arterial oxygen saturation monitoring was done in all the patients. Procedure-related mortality was 18.2%. Significant acute hemodynamic improvement was seen in the survivors (pre-balloon atrial septostomy cardiac index 1.88+/-0.48 L/min/m2; post-balloon atrial septostomy cardiac index 2.18+/-0.37 L/min/m2, p<0.009). Patients were followed up for a mean period of 20.3 months after the procedure (range: 3 months-5 years). There was functional improvement and increased exercise tolerance in all the patients for a mean follow-up period of 14.6 months (NYHA functional class 3.62+/-0.69 to 2+/-0.50). The estimated probability of survival in this cohort at 1 year was only 48%; but 7 of 8 patients (87%) who survived the procedure were alive at 1 year. CONCLUSION: We conclude that balloon atrial septostomy improves clinical status, hemodynamic variables and possibly also improves survival in selected patients with severe pulmonary artery hypertension. It remains a definite palliative option for refractory primary pulmonary hypertension. However, the procedure-related risks are high in very sick patients and, therefore, balloon atrial septostomy may be advocated early in the course of the disease.

Adult↗
PubMedDate not supplied

Management of cardiomyopathy resulting from incessant supraventricular tachycardia in infants and children.

BACKGROUND: Radiofrequency ablation is considered to be the treatment of choice in patients with ventricular dysfunction related to incessant supraventricular tachycardia. However, reservations regarding its use in infants and children prompted us to try alternative strategies for this group. METHODS AND RESULTS: Eight children (age range: 1 day to 10 years) were diagnosed to have tachycardia-related ventricular dysfunction in the past 6 years. They presented with symptoms of palpitation, dyspnea and/or generalized swelling over the body of 3 months to 2 years'duration. The cardiothoracic ratio at presentation was 64% (52%-70%) and ejection fraction was 22.2% (15%-45%). In 7 patients tachycardia was diagnosed to be ectopic atrial and in 1 it was permanent junctional reciprocating tachycardia. Six of these children were managed with intravenous/oral amiodarone in combination with digoxin (3) and/or propranolol (2). In one child addition of amiodarone to digoxin and propranolol led to polymorphic ventricular tachycardia, and amiodarone was withdrawn. Only one child underwent radiofrequency ablation as the first choice because regular follow-up was not possible due to logistic reasons. Sinus rhythm with normalization of ventricular function was achieved in 6 of the 7 children treated medically. One child continued to have frequent episodes of tachycardia and underwent successful radiofrequency ablation of a high right atrial ectopic focus. Two out of the 6 patients on amiodarone could be managed with only digoxin and propranolol after their ventricular function had returned to normal. A third patient relapsed on stopping amiodarone and underwent successful radiofrequency ablation of a left atrial ectopic tachycardia. CONCLUSIONS: Short-term amiodarone in combination with digoxin/propranolol is a safe and effective treatment strategy for infants/children with tachycardiomyopathy. Control of tachycardia is achieved in the majority, leading to recovery of ventricular function. This approach may avoid unnecessary ablations in children or at least postpone it till the procedure would be safer.

Amiodarone↗
PubMedDate not supplied

Ten-year experience with the arterial switch operation.

BACKGROUND: Arterial level repair is considered the most appropriate procedure for transposition of the great arteries. This report describes our experience with the arterial switch operation over the past decade. METHODS AND RESULTS: From January 1991 to January 2001, a total of 299 patients underwent an arterial switch operation for transposition of the great arteries or double-outlet right ventricle. Group I (n=169, 56.5%) comprised patients with transposition of the great arteries in whom the ventricular septum was essentially intact. Group II patients (n=130, 43.5%) had transposition of the great arteries with an additional significant ventricular septal defect or had double-outlet right ventricle with a subpulmonic ventricular septal defect. Of the total, 245 (82%) were males and 54 (18%) were females. In group I, the ages ranged from 2 days to 18 years (median 19 days) and weight ranged from 1.7 to 68 kg (median 2.5 kg). In group II, the ages ranged from 4 days to 4 years (median 90 days) and weight ranged from 2.5 to 17 kg (median 4 kg). Fifteen percent of the patients (25/169) in group I and 30% of the patients (39/130) in group II had features of bacteriologic infection. Arterial switch operation was performed on standard lines. In group I, 141 patients (83.4%) had a primary arterial switch operation while 28 (16.6%) underwent a rapid two-stage repair. Twenty-three patients required concomitant relief of associated anatomic left ventricular outflow tract obstruction. Operative mortality was 8.8% (15/169) in group I and 33% (44/130) in group II with an overall mortality of 19% (59/299). The major causes of operative mortality included pulmonary arterial hypertensive crisis (n=21), sepsis and related complications (n=16), and left ventricular failure (n=8). Coexisting arch anomalies, longer cross-clamp time, late presentation, and preoperative bacteriologic infections were incremental risk factors. Follow-up ranged from 1 to 10 years and was 87% complete. On follow-up, 91% of the patients were asymptomatic and off all medications. There were 3 late deaths and 5 patients required reoperation. CONCLUSIONS: Excellent long-term results are obtained in operative survivors following the arterial switch operation. However. operative mortality remains a concern in our set-up.

Adolescent↗