Interventional cardiology and cardiac surgery in India.

In India, as is happening world wide, the use of coronary interventions is likely to maintain the pace of growth seen in the past decade. This will be facilitated by better access to experienced cardiologists and catheterisation laboratories across the country. Similar growth has occurred in cardiovascular surgery despite constraints of infrastructure and affordability. Cardiovascular research established four decades ago is being carried forward with provision of indigenous technology equal to the best in the world, but tailored to local need and affordability.

Impact of a comprehensive telephone-based disease management programme on quality-of-life in patients with heart failure.

BACKGROUND: Disease management programmes for patients with heart failure have improving the quality-of-life (QOL) of patients with heart failure. METHODS: Patients attending the heart failure clinic were randomized into 2 groups of 25 patients each. The control group was managed in the heart failure clinic and the intervention group underwent the following additional interventions: (i) interactive sessions with the patient and spouse informing them about the disease, drugs, and self-management of fluid intake and diuretic dose; (ii) a telephonic helpline was established and regular telephone calls made to reinforce the information and modify drug dosages. The QOL was assessed using the Kansas City Cardiomyopathy questionnaire. Functional capacity was assessed by the 6-minute walk test. Continuous variables were compared with the Student t-test (paired or unpaired). RESULTS: There was significant improvement in the QOL and functional capacity of patients in the intervention group compared with controls over a 6-month period. The mean (SD) QOL scores in the intervention group improved from 60.0 (23.6) to 76.3 (17.3) but did not change significantly in the control group (62.2 [22.6] to 63.4 [21.9]). There was a similar improvement in the functional capacity measured by the 6-minute walk test in the intervention group (from 202.2 [81.5] to 238.1 [100.9] metres, p < 0.05) but not in the control group (193.8 [81.5] to 179.7 [112.0] metres). In the intervention group, the use of beta-blockers and angiotensin-converting enzyme inhibitors was similar but in the intervention group patients were placed on higher doses. There was no significant difference in the number of emergency room visits or admissions in either group. For every 20 patients in the intervention group, 14 patients improved by 1 functional class while in the control group this was observed in only 3 patients for every 20 treated. CONCLUSION: This study demonstrates that in the setting of a developing country, improvement in QOL by intensive management of heart failure patients through a heart failure programme with telephonic reinforcement and a helpline is greater than that usually achieved with drug therapy in a routine heart failure clinic.

Fatal atypical mycobacterial infection in a cardiac transplant recipient.

A 37-year-old female underwent heart transplantation for giant cell myocarditis. The patient died within three-and-a-half months of cardiac transplantation. Postmortem specimens from the heart and lung showed multiple necrotizing granulomas with numerous acid-fast bacilli. Polymerase chain reaction done on both the postmortem samples confirmed the presence of atypical mycobacterial infection. This fatal case of atypical mycobacteriosis in a cardiac transplant patient is reported for its rarity.

One and a half ventricle repair with pulsatile bidirectional Glenn: results and guidelines for patient selection.

BACKGROUND: The guidelines for performing a one and a half ventricle repair with pulsatile bidirectional Glenn remains controversial. This retrospective report summarizes the experience of a single institution, with an attempt at providing an answer. METHODS: Fifty consecutive patients, aged 4 months to 42 years, underwent intracardiac repair along with a superior cavopulmonary connection. Twenty-seven of the patients had had previous surgical palliation. Repair consisted of patch closure of the ventricular septal defect (n = 25), tricuspid valve repair (n = 26), reconstruction of the right ventricular outflow tract (n = 34), transpulmonary annular patch (n = 34), right ventricle to pulmonary artery homograft conduit (n = 4), and concomitant repair of atrioventricular canal (n = 9). Ten patients were left with a fenestration in the atrial septum. RESULTS: There were six hospital deaths (12%) and two late deaths (4.5%). Forty-two survivors were followed from 8 months to 116 months. Eighty-eight percent are in functional class I. Actuarial survival at 97 months was 74%. CONCLUSIONS: Moderate right heart hypoplasia constitutes a safe anatomic category for a pulsatile bidirectional Glenn. It is advisable not to proceed with a one and a half ventricle repair if postoperative residual pulmonary artery hypertension is anticipated. Patients requiring an intricate intracardiac repair and those with concomitant right heart hypoplasia may be better suited for a Fontan type of repair to reduce the complexity of the procedure.

Unusual totally anomalous pulmonary venous connection in right isomerism and functionally univentricular heart.

We report a rare variation in the pattern of totally anomalous pulmonary venous connection in that two vertical veins drained into the left brachiocephalic vein from a common pulmonary venous confluence. The child had associated right isomerism and functionally univentricular heart. Awareness of this possibility may avoid a reoperation or even death.

Mitral valve replacement with chordal preservation in a rheumatic population.

BACKGROUND AND AIM OF THE STUDY: Mitral valve replacement (MVR) with preservation of the subvalvular apparatus may maintain postoperative left ventricular (LV) function better than conventional MVR. A prospective study was undertaken to assess the advantages of chordal preservation in rheumatic patients undergoing isolated MVR. METHODS: Between January 1996 and January 1999, 240 patients with rheumatic etiology and normal LV ejection fraction (>50%) underwent isolated MVR using a St. Jude Medical prosthesis. Patients were allocated to two groups: in group I (n = 168), both the anterior and posterior chordae were preserved; in group II (n = 72), the entire native mitral valve apparatus was excised. Patients from both groups were evaluated postoperatively (mean 12.5 months) by echocardiography and treadmill testing. RESULTS: Demographic and clinical profiles were comparable in both groups. There were three early deaths in group I, and one in group II. There were no late deaths. One patient in group I required reoperation for a stuck mitral prosthesis. LV function (ejection fraction >50%) was better in group I (94%) than in group II (82%) (p <0.05). No patient in either group had LV outflow tract obstruction on echocardiography. Patients in group I showed better exercise performance on treadmill (Bruce protocol): 92% of group I patients versus 88% of group II patients (p >0.05) completed stage I; 16% of group I patients, but no group II patients, were able to complete stage IV (p <0.05). CONCLUSION: Mitral valve replacement with preservation of the subvalvular apparatus maintains LV function, and does not cause LV outflow tract obstruction.

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.

Univentricular repair in children under 2 years of age: early and midterm results.

BACKGROUND: Encouraging results have been obtained from early univentricular repair. Our study was designed to assess the impact of young age (less than 2 years) on the outcome of univentricular repair. PATIENTS AND METHODS: Between January 1992 and December 1998, 65 out of 311 patients undergoing univentricular repair at the All India Institute of Medical Sciences, New Delhi, were less than 2 years of age. We compared these 65 carefully selected patients (group 1) with the 246 patients who were more than 2 years of age (group 2). Since 1994, all patients of both groups had a fenestration of the intra-atrial baffle. RESULTS: The early mortality rate was 9.2% in group 1 and 7.7% in group 2. The overall Fontan failure rate was 12.3% in group 1 and 12.2% in group 2. In group 1, a higher incidence of Fontan failure was noted in patients with suboptimal weight, non-tricuspid atresia morphology, nonfenestrated Fontan and those who did not comply with more than two of Choussat's criteria, but the values were not statistically significant. Aortic cross-clamp time of more than 60 min was the only and highly significant predictor of Fontan failure (P < 0.01). The overall effusion rate was 24.05% in group 1 and 27.98% in group 2. In group 1, patients weighing less than 10 kg (P = 0.0007), without fenestration of the atrial baffle (P < 0.05) and with systemic ventricular dysfunction (P < 0.001), systemic ventricular end-diastolic pressure of more than 12 mmHg (P < 0.001), mean pulmonary artery pressure of more than 15 mmHg (P < 0.001) and aortic crossclamp time of more than 60 min (P < 0.01), were all found to be significant risk factors of pleural effusion. Pulmonary artery distortion needing reconstruction did not increase the Fontan failure or effusion rates. Oxygen saturation ranged from 85 to 94% (mean 89%) in patients having a functioning fenestration at a mean follow-up period of 30 months. The actuarial survival at 84 months was 90 +/- 0.04% in group 1 and 88 +/- 0.02% in group 2. CONCLUSIONS: Our results suggest that carefully selected patients under 2 years of age are suitable candidates for one-stage univentricular repair and that survival is not significantly different from that of older patients. Routine fenestration of the intra-atrial baffle is an option available to ameliorate morbidity and obviate mortality. Non-compliance with more than two of Choussat's criteria appears to be additive in unsatisfactory outcome.

Subaortic membrane excision: mid-term results.

BACKGROUND: Subaortic membrane (SAM) is a form of fixed subaortic obstruction in which a fibrous membrane is located below the aortic valve. AIM: To determine the role of surgical treatment for patients with a discrete SAM. PATIENTS AND METHODS: The hospital records of 45 patients (age range: 2-23 years; median 8 years) undergoing surgery for SAM between 1990 and 1998 at the All India Institute of Medical Sciences, New Delhi, India, were analysed. Preoperative echocardiographically calculated gradients across the left ventricular outflow tract ranged from 50 to 154 mmHg (mean: 86.5 +/- 33.2 mmHg). Nine patients had trivial aortic regurgitation (AR), 10 had mild AR and five had moderate-severe AR. The left ventricular ejection fraction (LVEF) ranged from 20 to 68% (mean 48 +/- 15%). Nineteen patients had significant left ventricular dysfunction (LVEF <50%). Transaortic resection of SAM was done in all patients along with excision of a wedge-shaped segment of septal muscle underlying the membrane. RESULTS: There were no early or late postoperative deaths. On follow up (up to 113 months), only four patients had gradients >30 mmHg. LVEF improved to 45-70% (mean 58 +/- 7.7%). AR reduced to mild in four patients and trivial in four patients, and did not progress further. CONCLUSION: Resection of SAM carries long-term benefits. Routine septal myectomy appears to be associated with a low risk of recurrence.

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.

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.

Right ventricular outflow tract after non-conduit repair of tetralogy of Fallot with coronary anomaly.

BACKGROUND: A total of 25 patients with tetralogy of Fallot and an important coronary artery crossing the right ventricular outflow tract underwent complete repair without use of an extracardiac conduit between January 1990 and December 1994. Repair was exclusively done by the transatrial or transatrial-transpulmonary approach. Age of these patients ranged from 1 to 12 years (mean 3.6 years). Three of the patients had already received a systemic to pulmonary artery shunt. METHODS: All patients reporting for follow-up (n = 18) were subjected to transthoracic echocardiography and, if required, cardiac catheterization and angiography. Right ventricle to pulmonary artery gradients were noted preoperatively, at discharge following repair and at follow-up study. RESULTS: Mean follow-up was 40.6 months (24 to 62 months). Mean early postoperative gradient was 23.5+/-13.4 mm Hg and 4 patients had significant (> 30 mm Hg) gradients. Mean late postoperative gradient was 20.6+/-12.4 mmHg and 2 patients had gradients greater than 30 mmHg. All the patients were in New York Heart Association functional class I at the time of last follow-up. CONCLUSIONS: Acceptable gradients across the right ventricular outflow tract are achievable following repair of tetralogy of Fallot in the presence of anomalous coronary artery across the right ventricular outflow tract using the transatrial or transatrial-transpulmonary approach. Most gradients were found not to vary significantly on subsequent follow-up.

Non-myxomatous cardiac tumours: twenty-year experience.

Eighty-eight patients underwent surgery for various cardiac tumours from January 1978 to June 1998 at our Institute. Seventy-seven tumours were myxomas, 10 were non-myxomatous and one was secondary cardiac tumour. Case records of the patients with non-myxomatous primary cardiac tumours and one secondary tumour were reviewed. Six of these primary tumours were benign and four, malignant. Age of the patients ranged from 26 days to 47 years. Among patients (3 children, 8 adults) with non-myxomatous primary cardiac tumours, dyspnoea on exertion was the commonest symptom and was the cause of presentation in seven out of 11 patients. Of the eight adults, six were in New York Heart Association functional class II/III and two in class IV. Echocardiographic diagnosis was possible in all the patients. Complete excision of the tumour was possible in all benign and two of the four malignant tumours. Incomplete resection was done in the secondary tumour. Of the six benign tumours, three were rhabdomyomas and one each of fibroma, haemangioma and lipoma. The malignant tumours were one each of fibrosarcoma, angiosarcoma, unclassified sarcoma and malignant mesothelioma. The secondary tumour was a malignant thymoma. Follow-up ranged from 1 to 10 years (mean 7.2 years). Of the patients with benign tumours, four out of six are alive; one patient died on the first post-operative day and one lost to follow-up. Two of the four patients with malignant cardiac tumours died, one was lost to follow-up and one is alive two years after surgery. The patient with secondary malignant thymoma to the superior vena cava was lost to follow-up three months after an uneventful recovery from surgery.

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.

Univentricular repair: is routine fenestration justified?

BACKGROUND: A decade after the introduction of baffle fenestration, the outcome of Fontan-type repair for hearts with a functional single ventricle finally looks promising. Our study was designed to assess the impact of fenestration on the outcome of univentricular repairs. METHODS: From January 1988 to December 1997, 348 patients (104 with tricuspid atresia and 244 with other morphological diagnoses) underwent univentricular repair at our institute. Since 1994, routine fenestration of the atrial baffle was performed in all patients (n = 126). RESULTS: The overall Fontan failure rate was 14% (50 of 348) and included 45 early deaths and five Fontan take downs. Absence of fenestration was the only and highly significant predictor of Fontan failure (risk ratio [RR] 3.3, 95% confidence interval [CI] 1.49 to 7.31, p = 0.002). Significant pleural effusion was seen in 27% of patients. Absence of fenestration of the atrial baffle (RR 3.97, 95% CI 2.17 to 7.26, p < 0.001) and aortic cross-clamp time more than 60 minutes (RR 2.15, 95% CI 1.3 to 3.5, p = 0.002) were found to be significant risk factors. The follow-up ranged from 6 to 120 months (mean 46.0 +/- 18.0 months). There were 12 late deaths and 5 patients were lost to follow-up. Actuarial survival (Kaplan Meier) at 90 months was 81% +/- 4%. Two hundred and fifty-eight patients (90%) were in New York Heart Association class I at their last follow-up visit. Oxygen saturation in the fenestrated group ranged from 85% to 94% (mean 89%). Thirty patients (26%) had spontaneous closure of the fenestration over a mean period of 34 months, and there has been no incidence of late systemic thromboembolism. In no instance has there been a need to close the fenestration. CONCLUSIONS: Elective fenestration of the intraatrial baffle is associated with decreased Fontan failure rate and decreased occurrence of significant postoperative pleural effusions. Routine elective fenestration of the atrial baffle may, therefore, be justified in all univentricular repairs.

Management of tetralogy of Fallot with absent pulmonary valve: early and mid-term results of a uniform approach.

The operative management of absent pulmonary valve syndrome remains controversial regarding palliative or one-stage correction, the need for pulmonary valve implantation and pulmonary arterioplasty. This retrospective report summarises the experience of a single centre with a view to provide some answers to this controversy. Forty-six consecutive patients including five infants, aged 2 months to 43 years, underwent primary surgical correction during the last 8.5 years. All the patients underwent two-dimensional echocardiography and cardiac catheterisation. Nine patients had mild and 10 moderate pulmonary artery hypertension. Repair consisted of patch closure of the ventricular septal defect and reconstruction of the right ventricular outflow tract. A valve was incorporated in the pulmonary position in 19 patients. Pulmonary arterioplasty was performed only in infants. Overall hospital mortality was 4 out of 46 patients (8.6%). Two out of five infants died accounting for 40 percent mortality. Forty-two survivors were followed up from 4 to 101 months; 40 patients are in functional class I and two in class II. Actuarial survival at 8.5 years was 91 percent. It is concluded that reconstruction of the right ventricular outflow tract with a transannular patch is sufficient in majority of patients. A selective approach to pulmonary valve insertion is recommended in patients with pulmonary hypertension or other anomalies. Pulmonary arterioplasty should be performed as the primary treatment in infants.

Right pulmonary artery left atrium communication.

A 12-year-old boy presented with central cyanosis, clubbing, and ejection systolic murmur in the left second and third intercostal space. Chest roentgenogram showed a round opacity on the right border of cardiac silhouette. Echocardiography revealed a communication between right pulmonary artery and left atrium, which was later confirmed on selective pulmonary cineangiography. Successful surgical ligation without cardiopulmonary bypass resulted in the cure of the disease.