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.

Univentricular repair. Early and midterm results.

A total of 202 patients (62 with tricuspid atresia and 140 without tricuspid atresia) underwent univentricular repair at our unit from January 1990 to September 1994. Of these patients, 182 had nonfenestrated and 20 had fenestrated interatrial baffles. Early mortality was 15.9% (29/182) in the group with nonfenestrated baffles and 5% (1/20) in the group with fenestrated baffles. The follow-up period ranged from 2 to 58 months. Seven late deaths occurred, and five patients were lost to follow-up. Of 160 patients who have been evaluated in the outpatient department in the past 3 months, 142 (88.75%) required no cardiac medicines and were in functional class I. Risk factors analyzed for early mortality and significant effusion were age, preoperative diagnosis, type of Fontan modification, cardiopulmonary bypass time, aortic crossclamp time, pulmonary artery size, associated pulmonary arterioplasty, takedown of systemic-pulmonary artery shunt, and pulmonary artery debanding, along with the Fontan operation. Bypass time exceeding 120 minutes was associated with a higher early mortality (12/47 vs 18/155; p = 0.0187). Bypass time exceeding 120 minutes (p = 0.0456) and aortic crossclamp time exceeding 60 minutes (p = 0.0278) were associated with significant postoperative effusion. Other factors were not associated with any significantly increased risk for early mortality or postoperative effusions. Fenestration of the interatrial baffle appeared to decrease early mortality, although the numbers are too small to be statistically significant. The prevalence of effusions did not differ significantly between the group with fenestrated baffles and the group without fenestrated baffles.

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.

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.

Malignant thymoma presenting as intracardiac tumor and superior vena caval obstruction.

A case of malignant thymoma with intracardiac infiltration and intrinsic superior vena caval obstruction due to the tumor is reported. Intracardiac invasion by a thymoma is rare and for this reason is believed worthy of a report.

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.

Aneurysm of sinus of Valsalva dissecting into interventricular septum.

BACKGROUND: Dissection of interventricular septum by aneurysm of the sinus of Valsalva is extremely rare. We present our experience with the management of 10 patients with this condition. METHODS: Ten patients with aneurysm of the sinus of Valsalva dissecting into the interventricular septum were managed at All India Institute of Medical Sciences, New Delhi, between May 1987 and September 1996. Conduction abnormalities and aortic insufficiency dominated the clinical picture. Eight patients underwent surgical repair. Two patients refused operation, and only permanent pacemaker implantation was done for complete heart block in both these patients. RESULTS: There was no hospital mortality. Follow-up ranged from 1 to 9 years. There was one late death due to carcinoma of the larynx, and 1 patient required reoperation for persistent aortic insufficiency. All other patients who underwent operation are in New York Heart Association functional class I. CONCLUSIONS: We recommend surgical repair of this condition to deal with aortic regurgitation and to avoid the potential risk of rupture, thromboembolism, and infective endocarditis. However, surgical repair offers no guarantee against arrhythmias and conduction abnormalities.

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.

Surgical experience with intracardiac myxomas: long-term follow-up.

BACKGROUND: Myxomas are the most common benign intracardiac tumors. This report summarizes our 20-year experience with these tumors. METHODS: Sixty-six patients (25 male) with a median age of 39 years (range, 6 to 70 years) underwent surgical excision of primary or recurrent intracardiac myxomas during the years 1976 to 1996. Symptom duration ranged from 2 to 8 months. There were 55 left atrial myxomas, 10 right atrial myxomas, and 1 biatrial myxoma. Three of the patients were in one family. The surgical approach comprised complete wide excision. RESULTS: There were two early deaths. Late follow-up is 89% (57/64) complete. There was one late death, which was not due to a cardiac cause. Echocardiography at a mean follow-up of 66.9 months (range, 7 to 241 months) showed no recurrence of sporadic myxomas. However, 2 of the 3 patients with familial myxomas had recurrence. CONCLUSIONS: Surgical excision of atrial myxoma gives excellent short-term and long-term results leading to eventual cure of nonfamilial myxomas. However, familial myxomas retain a strong tendency to recur even 20 years after excision.

Repair of total anomalous pulmonary venous connection in infancy: experience from a developing country.

BACKGROUND: Corrective surgery for total anomalous pulmonary venous connection in infancy still carries high morbidity and mortality rates in developing countries. The present study evaluates the factors responsible for it. METHODS: Seventy-three infants were operated on for total anomalous pulmonary venous connection from January 1987 through October 1997. Age ranged from 5 days to 12 months (mean, 3.9+/-0.24 months), with 10 (13.7%) patients younger than 1 month old. Patient weight varied from 2.0 to 5.2 kg (mean, 3.7+/-0.27 kg). Most (90.5%) patients were small for their ages (< 50th percentile). Anomalous connection was supracardiac in 42 (57.5%), cardiac in 18 (24.7%), infracardiac in 4 (5.5%), and mixed in 9 (12.3%) patients. Thirty-five patients had obstructed drainage. Preoperatively, 30 patients received antibiotic therapy for respiratory tract infection, 3 patients had balloon atrial septostomy, and 4 patients required mechanical ventilation. Fifteen patients (20.5%) were operated on as an emergency procedure. For supracardiac and infracardiac connections, a posterior approach was used for anastomosis. In cardiac type, coronary sinus was unroofed and the resultant defect along with atrial septal defect was closed with a single patch. RESULTS: The operative mortality rate was 23.3% (17 of 73). Pulmonary hypertensive crisis was the cause of death in 10 patients. Emergency operation and weight less than the 25th percentile were the important risk factors for operative mortality. Young age (< 1 month) and type of drainage did not affect the mortality. Follow-up ranged from 1 to 108 months (mean, 56.4+/-26.0 months). There were two late deaths. The actuarial survival (Kaplan Meier) at 9 years was 72.87%+/-5.39%. CONCLUSION: Failure of early recognition, and thus delayed referral, accounted for onset of cardiac cachexia, respiratory tract infection, and severe pulmonary hypertension, which had a major effect on unfavorable outcome.

The combination of a left aortic arch, a right-sided descending thoracic aorta with a left-sided arterial duct.

A nine-year-old male child presented with a history of recurrent chest infections and breathlessness. On investigation, he was found to have a left aortic arch with a right-sided descending thoracic aorta along with a left-sided arterial duct. He also had mild aortic stenosis with regurgitation. The duct was successfully ligated under controlled hypotensive anesthesia through a left posterolateral thoracotomy.

Aortico-right ventricular tunnel.

A 2-year-old child with aortico-right ventricular tunnel is reported for its rarity. The right coronary artery originated from the distal end of the tunnel. The frequent occurrence of coronary artery origin abnormality with this anomaly is highlighted.

Aortic atherosclerosis and perioperative stroke in patients undergoing coronary artery bypass: role of intra-operative transesophageal echocardiography.

Intra-operative transesophageal echocardiography was performed in 126 patients undergoing coronary artery bypass grafting. Significant protruding atheromas (grade IV and V; Katz et al., 1992) were present in 12 patients (9.5%). Protruding atheromas had significantly higher incidence in patients above 60 years in age. Preoperative assessment with chest roentgenography and angiography, as well as intra-operative assessment by surgical palpation proved to be insensitive in detecting aortic atheromas. Out of four patients with grade V atheromas, two (50%) developed right hemiplegia postoperatively. For the rest, patients with grade V atheromas and eight patients with grade IV atheromas, surgical technique was modified and that helped in preventing occurrence of perioperative stroke.

Total correction of tetralogy of Fallot in adults--surgical experience.

A total of 100 patients of tetralogy of Fallot aged 13 years and over were operated upon at the All India Institute of Medical Sciences, New Delhi, India between January 1991 and December 1996. There were 69 males (69%) and 31 females (31%). Age ranged from 13 years to 43 years (mean 19.66 years). Twenty % of patients had preoperative complications like haemoptysis, cerebrovascular accidents, brain abscess and infective endocarditis. Twenty-two patients had previous palliative shunts. Fifteen patients had coil embolisation of major collaterals prior to surgery. In hospital mortality rate was 4%. Follow-up ranged from 1 month to 5 years (mean 3.4 years). There was one late death due to infective endocarditis. Postoperatively 93.6% patients were in NYHA class I. Significant residual defects warranting re-operation were present in three patients. Total correction of tetralogy of Fallot in older patients can be performed with acceptable results.

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.

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.

Orthotopic pulmonary valve replacement with a homograft.

Eight pulmonary valve replacements (PVR) have been performed from January 1992 to October 1994. Three patients (mean age 7.7 years, range two to 16 years) had absent pulmonary valve with tetralogy of Fallot and underwent primary PVR at the time of surgical correction. Five other patients, who had correction of tetralogy of Fallot (four cases) and of double outlet right ventricle with ventricular septal defect and pulmonary stenosis (one case), were reoperated for pulmonary regurgitation with progressive right ventricular dysfunction. Mean age at the time of reoperation was 18 years (range seven to 34 years). There was no early death. Early postoperative recovery was satisfactory in all of them. The follow up ranges from six to 35 months (mean 19 months). Seven patients were in functional class I and one in functional class II when they were last evaluated in the out-patient department and five of them were off diuretics and vasodilator. In the presence of right ventricular dysfunction pulmonary regurgitation is poorly tolerated. A competent and non-obstructive pulmonary valve is often life saving in these critically ill patients.

Pathology of cardiac transplantation--the initial experience.

BACKGROUND: Cardiac transplantation has recently been started in India at the All India Institute of Medical Sciences, New Delhi. All transplants are monitored by right ventricular endomyocardial biopsies. This study describes our initial experience with the histopathological assessment of post-transplant endomyocardial biopsies. METHODS: Fifty-four endomyocardial biopsies from 8 transplanted hearts were reviewed. At least 3 haematoxylin-eosin and Masson trichrome stained sections were available on each biopsy. Special stains and immunohistochemistry were done as required. Biopsies were graded for rejection using the grading system of the International Society for Heart and Lung Transplantation. Infection, 'Quilty' effect, biopsy site, ischaemia and reperfusion injury were also assessed. RESULTS: There was no evidence of rejection in 34 biopsies. Eleven biopsies showed mild rejection. Only 9/54 biopsies from 4 of the transplant recipients had clinically significant grades of rejection. Biopsy sites were present in 13/54 biopsies. Ischaemia was present in the first biopsy after transplantation in 4 recipients. There was no evidence of viral or parasitic infection. 'Quilty' lesions were seen in 14/54 biopsies from 5 transplant recipients. Four of these 5 recipients had 'Quilty' lesions on more than 1 biopsy. CONCLUSIONS: The low rejection rate at our centre is consistent with reports from around the world. Endomyocardial biopsy has been a safe, convenient and useful method for diagnosing rejection and monitoring the postoperative course of cardiac transplants at our centre.

Aortic valve replacement with a homovital valve.

"Homovital" allografts (viable homografts) are a good substitute for native aortic valves. A case of aortic valve replacement with a homovital aortic allograft is reported along with the results of immunologic investigations. Postoperatively, there was no clinical or echocardiographic evidence of valve dysfunction, and immunologic tests did not show evidence of graft versus host reaction.