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.

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.

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.

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.

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.

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.

Post-infarction ischaemic mitral regurgitation: what determines the outcome.

Ischaemic mitral regurgitation is an important determinant of survival in patients with coronary artery disease. A retrospective analysis was performed to evaluate the overall outcome and its determinants in patients with ischaemic mitral regurgitation. Over a period of 10 years, 72 patients underwent operations for mitral regurgitation of ischaemic origin. Age ranged from 37 to 68 years (mean 54.6 +/- 10.4 years), and 62 (86.1%) were male. Thirteen (18%) patients had acute and 59 (82%) had chronic ischaemic mitral regurgitation. Twenty-one patients were in New York Heart Association class II, 32 in class III and 19 in class IV. Moderate to severe left ventricular dysfunction was present in 42 patients. Valve prolapse was present in 35 (48.6%) patients and restricted leaflet motion secondary to myocardial dysfunction was present in 37 (51.4%) patients. All the patients were operated using standard cardiopulmonary bypass technique. Mitral valve was replaced in 33 patients and repaired in 39. Repair included a combination of techniques: chordal transposition (n = 2), chordal shortening (n = 18), leaflet resection (n = 2), posterior collar annuloplasty (n = 35) and annuloplasty with flexible Duran's ring (n = 3). Operative mortality was 18.1 percent (13/72). Low cardiac output was the cause of death in the majority (n = 10). Acute presentation and presence of restricted leaflet motion were the significant predictors of early mortality. Follow-up ranged from 3 to 84 months (mean 41.6 +/- 10.2 months). Late mortality was 46.2 percent. Actuarial survival in operative survivors at five years was 44.4 +/- 8.8 percent. To conclude, ischaemic mitral regurgitation carries a poor early and late outcome, with left ventricular dysfunction and presence of restricted leaflet motion being important contributors to it. In addition, acute presentation also reflects greater early mortality.

Ambulatory 24-hour electrocardiographic monitoring following total cavopulmonary connection.

A total of 72 patients (53 males, 19 females) in the age range 1-22 years (mean 6.4 years) who underwent univentricular repair between April 1990 and May 1997 at our institute were followed up from six months to seven years (mean 3.6 years). Twenty-four hours ambulatory electrocardiographic Holter monitoring was done in all the patients. Thirty-four out of 40 patients (85%) who underwent fenestrated total cavopulmonary connection and 25 out of 32 patients (78%) who underwent non-fenestrated total cavopulmonary connection had normal Holter recording; thus 59 out of 72 (82%) patients had normal findings. Among the fenestrated total cavopulmonary connections, three patients each had atrial tachycardia and sinus bradycardia. In the non-fenestrated group, two patients had atrial tachycardia, three had supraventricular tachycardia, one sinus bradycardia and one had intermittent ventricular tachycardia. No correlation was found between age at surgery, pre-operative morphology, cross clamp time or cardiopulmonary bypass time with post-operative arrhythmia. To conclude, post-operative arrhythmias following total cavopulmonary connection were not related to age at surgery, pre-operative morphology, cross clamp or cardiopulmonary bypass time. However, a larger patient pool and longer follow-up is required for evaluation of any definitive correlation.

Adult cyanotic congenital heart disease: surgical experience.

Cyanotic congenital heart diseases constitute about 10 percent of total congenital heart disease cases in adults in the developing world. Prolonged cyanosis and old age adversely affect the outcome of surgery, thus posing a challenge to the cardiac surgeons. This study was conducted to assess the feasibility, safety and outcome of surgery in this group of patients. From January 1991 to December 1997, a total of 303 patients, aged 14 to 54 years (mean 19.8 +/- 1.5 years) with diagnosis of various cyanotic congenital heart diseases were operated at our institute. There were 210 males (69.3%). Two hundred and forty-seven patients (81.5%) had tetralogy of Fallot's physiology, 51 patients (16.8%) had single ventricle physiology and five (1.6%) had other lesions. Sixty-six patients (21.7%) had pre-operative complications such as haemoptysis, epistaxis, cerebrovascular accidents, brain abscess and infective endocarditis. Sixty patients (19.8%) had previous palliative shunts and 26 patients (8.5%) had coil embolisation of major aortopulmonary collaterals prior to surgery; 229 patients (75.5%) underwent biventricular repair, 52 (17.1%) had univentricular repair, 22 (7.7%) had palliative shunts and one patient had open ligation of a major aortopulmonary collateral in addition. In-hospital mortality was 3.3 percent. Follow-up period ranged from five months to seven years (mean 4.2 +/- 1.8 years). There were two late deaths. Of the 291 survivors, 11 were lost to follow-up. Two hundred and fifty-eight patients (92.1%) are in New York Heart Association class I. Significant residual defects warranting reoperation were present in four patients (1.3%). It is concluded that congenital heart surgery in older cyanotic patients can be performed safely with satisfactory results.

Appraisal of histogenesis of cardiac myxoma: our experience of 78 cases and review of literature.

Cardiac tumours are uncommon. Of these, myxomas have generated significant interest, not only because it is the commonest cardiac neoplasm but also because of controversial theories regarding its histogenesis. We encountered 78 cases of cardiac myxoma in our centre between 1976 and 1997. These included 73 sporadic cases and five cases from a single family of mother, daughter and son. The familial cases had recurrent, biatrial cardiac myxomas. Histopathologic analysis, immunohistochemical study, electron microscopic evaluation and DNA ploidy analysis done in some of these cases revealed evidence in support of the neoplastic nature of this entity. This communication sums up our observations and literature related to the histogenesis of cardiac myxoma.

Routine cleft closure in repair of complete atrioventricular septal defects.

Thirty patients with complete atrioventricular septal defect were operated upon between 1989 and 1996 at our institute. Their ages ranged from two months to 24 years (mean age 2.81 years). All patients had severe pulmonary arterial hypertension except one who had associated pulmonic stenosis. Four patients had severe atrioventricular valve regurgitation and another six had moderate regurgitation. Five patients had Down's syndrome. The cleft in the left atrioventricular valve was closed in all but seven patients. There were five (16.66%) in-hospital deaths which included two patients in whom the cleft was left open. Follow-up ranged from three months to eight years. All patients were evaluated by 2D echocardiography in the immediate post-operative period and three to six monthly subsequently. One patient progressed to moderate and another to severe left atrioventricular valve regurgitation during follow-up. In both the patients the cleft had been left open. All other patients continue to have stable left atrioventricular valve status, that is, none or trivial to mild regurgitation. In view of late deterioration of the left atrioventricular valve regurgitation only in those patients where the cleft was left open, we suggest complete closure of the cleft in all complete atrioventricular canal defects.

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.

Aortoaortic bypass: indications, techniques and results.

The standard surgical treatment of complex cases of aortic obstructions is difficult and sometimes even hazardous, thus necessitating the use of alternative surgical methods to manage these cases. Between 1986 and 1995, nine such patients underwent ascending aorta to descending/abdominal aorta bypass graft as an alternative procedure at a premier medical institution. There was no hospital death nor any significant morbidity. Preoperative systolic blood pressure in right upper limb ranged from 150 mm Hg to 230 mm Hg (mean 180.5 mm Hg) while postoperative systolic blood pressure in right upper limb ranged from 126 mm Hg to 150 mm Hg (mean 134 mm Hg), thereby showing marked improvement. Preoperative pressure gradient across the aortic obstruction ranged from 50 mm Hg to 120 mm Hg (mean 87.2 mm Hg). It was relieved in all except two patients who had resting gradients of 10 mm Hg and 12 mm Hg respectively. All the patients were relieved of their symptoms. After a mean follow-up of 33.3 months (range 6 to 108 months), all the patients are in New York Heart Association (NYHA) class I with evidence of good distal perfusion. This technique of bypassing the aortic obstruction has the added advantage of avoiding the complications associated with standard technique.

Serial echocardiography in transplanted heart: an initial report of five cases.

Role of echocardiography including Doppler is established in selection of donor and in the care of patients after cardiac transplantation. Its value for recognition of transplant rejection is however still evolving. We present here, serial echocardiographic findings in five male patients, aged 22 to 46 years who underwent cardiac transplantation at our institution. There was no appreciable change on follow-up in the left ventricular dimensions, thickness of left ventricular posterior wall and interventricular septum and left ventricular systolic function. Transient pericardial effusion was noted in two patients. Trivial to mild mitral and tricuspid regurgitation was observed. The diastolic function of the left ventricle, as assessed by mitral valve inflow wave pattern, was normal in four patients. However in the fifth patient, there was evidence of diastolic dysfunction of the left ventricle and this change was accompanied by rejection episode (IIIB changes) in the endomyocardial biopsy. With resolution of rejection in biopsy, the diastolic function by Doppler also returned to normal. We conclude that echocardiography has a definite role in the general care of patients after cardiac transplantation. Transient small pericardial effusion and mild atrioventricular valve regurgitation are common after the operation. Echocardiography may also be of use in recognition of rejection episode by demonstrating evidence of diastolic dysfunction of the left ventricle. Currently, endomyocardial biopsy remains the gold standard for diagnosis of rejection.