Thomas Titus

Prognosis for patients with Eisenmenger syndrome of various aetiology

The objective of this study was to determine the long-term survival pattern and variables affecting long-term survival and complications occurring during follow-up of patients with Eisenmenger syndrome. A retrospective study of patients diagnosed with Eisenmenger syndrome were followed up. A tertiary care centre was used and it provided superspeciality services in various disciplines. The subjects included 201 patients with Eisenmenger syndrome--diagnosed by a combination of echocardiography and a peripheral arterial oxygen saturation study and/or cardiac catheterisation with or without angiocardiography--worked up and followed up for variable duration over a period of 16 years from 1976 to 1992. One hundred nine patients were females and 92 were males--age of presentation varied from 3 months to 62 years (mean +/- standard deviation 19.23 +/- 12.62 years). A total of 12 different anatomic lesions were seen--the most common three being ventricular septal defect (33.33%), aterial septal defect (29.85%), and patent ductus arteriosus (14.23%). History, physical examination, chest skiagram and electrocardiogram established only the presence of pulmonary arterial hypertension except where differential cyanosis indicating ductus was discernible or the degree of splitting of second heart sound provided some clue to the level of shunt. Contrast echocardiography, completed in 25.4% established the level of shunt in all patients. In others the diagnosis was confirmed by cardiac catheterisation. Twenty patients died during a mean follow-up period of 54.6 +/- 54.47 months. Sudden cardiac deaths (30%), congestive heart failure (25%) and haemoptysis (15%) were the most predominant causes of death. Only one patient died during puerperium.(ABSTRACT TRUNCATED AT 250 WORDS)

Coronary artery fistula in children and adults: a review of 25 cases with long-term observations

We studied 25 patients with coronary artery fistula between 1976 and 1994. Age ranged from 1 to 58 years. Twelve patients were symptomatic; seven had dyspnoea, four had angina, one had palpitation and one had syncope. Coronary arteries in four. Coronary artery fistula drained into right ventricle in 11, right atrium in nine, pulmonary artery in four and left ventricle in two. The Qp/Qs ranged from 1.0 to 2.6 with a mean of 1.39 +/- 0.38. Five patients had associated cardiac anomalies. Two had atrial septal defects, one had patent ductus arteriosus, one had atresia of proximal right coronary artery and in one patient, the right coronary was arising from left coronary artery. Five patients underwent surgery without any operative mortality. Thirteen patients were followed-up medically for a mean period of 6.1 +/- 5.1 years. There were no complications related to coronary artery fistula during follow-up. In one patient coronary artery fistula closed spontaneously.

Balloon angioplasty for native coarctation of the aorta in children and adults : factors determining the outcome

Balloon angioplasty was performed in 46 patients (age 2-40 yr) with discrete native coarctation of aorta. Patients with associated patent ductus arteriosus, aberrant subclavian artery and aneurysms were excluded. The peak systolic gradient across the coarcted segment decreased from 52.1 +/- 18.5 mmHg to 18.6 +/- 14.8 mmHg (p less than 0.001), and the diameter of the coarcted segment increased from 3.6 +/- 1.7 mm/m2 to 9.1 +/- 3.2 mm/m2 (p less than 0.001). Follow-up haemodynamic and angiographic studies performed in 21 patients at 13.1 +/- 6.9 months after angioplasty, showed good results in 15 patients. Four patients undergoing haemodynamic study and 4 other patients undergoing noninvasive evaluation were graded as having bad results at follow-up. In 5 of these patients the poor results were due to primary failure of angioplasty in relieving the gradient, and three developed re-coarctation after initial fall in the trans-coarctation gradient. Four risk factors were identified on univariate analysis, which were associated with significantly larger residual gradients at follow-up: (1) size of isthmus/size of coarcted segment ratio less than 3.0; (2) size of post-coarctation descending aorta/size of isthmus ratio greater than 1.75; (3) size of coarcted segment after angioplasty/size of coarcted segment before angioplasty ratio less than 2.0; and (4) size of balloon/size of coarcted segment ratio less than 3.0. The presence of one or more risk factors was associated with bad late results. On multivariate analysis the ratio of balloon size/coarcted segment size was found to be the sole independent predictor of the late outcome (p less than 0.02).(ABSTRACT TRUNCATED AT 250 WORDS)

Clinical profile and natural history of Ebstein's anomaly of tricuspid valve

There were 63 patients of Ebsteins anomaly of tricuspid valve encountered from 1976 to 1991; 28 (44.4%) were male and 35 (55.6%) female. Their age at presentation ranged from 3 months to 51 years. Five (7.9%) patients were asymptomatic, 48 (76.2%) had class II-III exertional dyspnoea, palpitation or both. Thirty patients (47.6%) had cyanosis. Electrocardiogram showed paroxysmal atrial fibrillation in two, chronic atrial fibrillation in four (6.3%), paroxysmal supraventricular tachycardia in seven, atrial or ventricular ectopic beats in five (7.9%), 2:1 atrioventricular block in one (1.6%), complete atrioventricular block in two (3.2%) and type B WPW syndrome in nine patients (14.3%). Chest X-ray showed diminished vascularity in 22 (34.9%). Diagnosis was established by cardiac catheterization and or echocardiography. Atrialized right ventricular chamber was demonstrated in 51 (80.9%) by angiography and in 40 (63.5%) by electrophysiology. Patients were followed up for 1-172 months. Seventeen patients (26.9%) required surgery. Three patients (4.8%) died during medical follow-up, and five (7.9%) died following surgery. Survival probability for 46 medical patients was 88.9% at 172 months. Factors affecting survival were pulmonary blood flow, cyanosis, clubbing and systemic arterial oxygen saturation.

Percutaneous transvenous mitral commissurotomy using an Inoue balloon in children with rheumatic mitral stenosis

Percutaneous transvenous mitral commissurotomy (PTMC) using the Inoue technique was performed in 557 patients with rheumatic mitral stenosis. Of these, 107 were children aged 10-18 years (mean +/- SD 14.5 +/- 2.3). All patients were symptomatic New York Heart Association (NYHA) Class II (n = 78) and Class III (n = 29). All were in sinus rhythm. Following PTMC, the mitral valve area (MVA) increased from 0.73 +/- 0.18 to 1.7 +/- 0.53 cm2 (P < 0.001). There was a significant fall in mean transmitral gradient from 15.6 +/- 5.2 to 5.1 +/- 2.3 mmHg, and in mean pulmonary artery pressure from 41 +/- 15 to 28.4 +/- 10 (P < 0.001). Cardiac tamponade developed in one patient. One patient developed severe mitral regurgitation requiring emergency mitral valve replacement. Five patients (4.7%) developed moderate mitral regurgitation. There was no mortality or cerebral embolism in any of the children. Four patients (3.7%) had oximetry evidence of atrial septal defect. Mean mitral valve area and transmitral gradient at 14 months mean follow up was 1.68 +/- 0.4 cm2 and 6 +/- 3.5 mmHg, respectively, and were comparable to the immediate post-PTMC results. Two patients (1.8%) developed restenosis. The immediate haemodynamic results in children were compared to 450 adult patients who underwent PTMC in the same period. The outcome was similar in both groups. Children were found to have significantly higher pulmonary artery pressure compared to adults. We found that PTMC using an Inoue balloon is very effective and safe in children, and consider that it should be the procedure of choice for young patients with symptomatic rheumatic mitral stenosis.

Balloon pulmonary valvoplasty: factors determining short- and long-term results

Balloon pulmonary valvoplasty was performed in 139 patients (age 2-44 years) with pulmonary valve stenosis. The right ventricular peak systolic pressure decreased from 137.1 +/- 46.8 mmHg to 76 +/- 51.3 mmHg (P < 0.001) and the right ventricle to pulmonary artery peak systolic gradient decreased from 116.3 +/- 49 mmHg to 54.4 +/- 51.9 mmHg (p < 0.001). There was no significant change in systemic artery systolic pressure. The right ventricular peak systolic pressure to systemic artery systolic pressure ratio decreased from 1.13 +/- 0.41 to 0.63 +/- 0.42 (P < 0.001). Patients with incomplete immediate relief of obstruction (right ventricle to pulmonary artery peak systolic gradient > 35 mmHg) had higher pre-dilatation right ventricular peak systolic pressure (161.1 +/- 45.3 mmHg vs. 93.9 +/- 38.8 mmHg, P < 0.001) and higher right ventricular peak systolic pressure to systemic artery systolic pressure ratio (1.31 +/- 0.42 vs 0.98 +/- 0.33, P < 0.001) pre-dilatation and were older (17.2 +/- 8.6 years vs. 12.8 +/- 9.7 years, P < 0.01). The residual right ventricle to pulmonary artery peak systolic gradients in the majority of patients were infundibular, which regressed at follow up even in patients who did not receive long-term oral beta blockers. Follow up catheterisation in 79 patients after 13 +/- 8.7 months showed a further fall in right ventricular peak systolic-pressure (P < 0.001) and right ventricle-to-pulmonary artery peak systolic gradient (P < 0.001).(ABSTRACT TRUNCATED AT 250 WORDS)

Percutaneous treatment of congenital coronary arteriovenous fistulas.

BACKGROUND Congenital coronary arteriovenous fistulas (CAVF), although rare, can present as significant hemodynamic lesions necessitating intervention. METHODS AND RESULTS Six patients (two males) with congenital coronary arteriovenous fistulas (CAVF) underwent percutaneous transcatheter occlusion. The ages ranged from 4 years to 49 years (mean 20.1 years). The fistulas had their origins from the right coronary artery (two), the left anterior descending coronary artery (two), and the left circumflex coronary artery (two). One of the fistulas drained to the right ventricle, four drained to the right atrium, and the remaining one to the left ventricle (LV). The fistulas were closed using the arterial approach with Cook™ coils in two patients and with nitinol ductal occluders (NDOs) using the venous approach in four patients. One patient developed dissection of the wall of the fistula during attempted closure and had spontaneous occlusion of the fistula. Complete occlusion of the fistulas were achieved in all patients. Complications consisted of migration and embolization of the coils in one patient (later closed successfully with NDO) and myocardial infarction occurring two weeks following successful closure in another patient. At mean follow-up of 39.6 +/- 22.9 months, all patients were asymptomatic and echo-Doppler evaluation revealed no residual fistulae. CONCLUSIONS CAVF are very well amenable to percutaneous closure with acceptable morbidity and high success rates.

Unusual Coarctation—the PHACE Syndrome: Report of Three Cases

OBJECTIVES To report the clinical features, nature of vasculopathy observed and the management instituted in three cases of PHACE syndrome. RESULTS All three patients were noted to have diffuse vasculopathy and aortic arch hypoplasia in addition to aortic coarctation adding substantial surgical risk or making surgery palliative. CONCLUSION PHACE syndrome should be considered in the differential diagnosis of aortic coarctation associated with aortic arch hypoplasia.

Doppler echocardiographic assessment of TTK Chitra prosthetic heart valve in the mitral position

AIMS TTK Chitra heart valve prosthesis (CHVP), a tilting disc mechanical heart valve of low cost and proven efficacy, has been in use for the last 15 years. Although various studies substantiating its long-term safety and efficacy are available, no study had assessed its echocardiographic characteristics. The purpose of this study was to determine the normal Doppler parameters of CHVP in the mitral position and to assess whether derivation of mitral valve area (MVA) using the continuity equation (CE) and more commonly used pressure half-time (PHT) method is comparable in the functional assessment of this tilting disc mitral prosthesis. METHODS AND RESULTS Doppler echocardiography was performed in 40 consecutive patients with CHVP in the mitral position. All patients were clinically stable, without evidence of prosthetic valve dysfunction such as significant obstruction or regurgitation, endocarditis, left ventricular dysfunction (ejection fraction <40%), or significant aortic regurgitation. Valve sizes studied included 25, 27, and 29 mm. Mitral valve area was derived both by the PHT method and by the CE, using the stroke volume measured in the ventricular outflow tract divided by the time-velocity integral of CHVP jet. The peak Doppler gradient ranged from 5 to 21 (mean 11.0) mmHg, and the mean gradient ranged from 1.7 to 9.2 (mean 4.1) mmHg. Mean gradient negatively correlated with an increase in the actual orifice area (AOA) derived from the valve orifice diameter given by the manufacturer (r = -0.45, P = 0.004). Mitral valve area calculated by both PHT and CE increased significantly with an increase in the AOA (r = 0.42, P = 0.007 and r = 0.32, P = 0.046, respectively). Mitral valve area by the CE averaged 1.55 +/- 0.36 cm(2) (range 0.85 cm(2) for a 25 mm valve to 2.41 cm(2) for a 29 mm valve) and was smaller than by PHT (mean 2.04 +/- 0.41 cm(2), range 1.40-3.14 cm(2); P = 0.0001; t-test), irrespective of whether PHT is less than or >110 ms. CONCLUSION The Doppler parameters obtained with CHVP in the mitral position are comparable with those obtained with the different prosthetic valves in common use. In the selected group of patients with CHVP, assessment of MVA by the PHT method is comparable with that by the CE. Areas by both methods were smaller than the AOA provided by the manufacturer, as seen in other similar design valves.

Central pulmonary artery anatomy in right ventricular outflow tract obstructions

We reviewed the cine-angiograms of 190 patients with right ventricular outflow tract (RVOT) obstructions for size and anatomy of pulmonary arteries, patent ductus arteriosus (PDA) and major aorto pulmonary collateral arteries (MAPCAs). Patients were grouped into three, Tetralogy of Fallot (TOF) with pulmonary atresia (group 1, N=86), TOF with pulmonary stenosis (group 2, N=97) and 7 cases of pulmonary atresia with intact interventricular septum (group 3). Out of 86 patients in group 1, 49 had PDA alone, 30 had MAPCAs alone, six had both and one had none. In group 2, 31 patients had persistent PDA and one patient had MAPCAS and PDA. A discrete stenosis (DS) of pulmonary artery was seen significantly more in patients with RVOT obstructions associated with PDA compared to patients without PDA (67/84 vs. 5/96). Out of the 84 cases with ducti, 53 had stenosis of the pulmonary artery at the site of ductus insertion. Thus presence of PDA was an important factor in the development of DS. The likely cause of pulmonary artery stenosis in TOF with PDA may be the opposing flows through RVOT and PDA producing a watershed effect at the ductus-pulmonary artery junction. Diffuse hypoplasia of pulmonary arteries (DH) was seen more significantly in RVOT obstructions associated with MAPCAs, compared to other patient groups (19/36 vs. 14/87). These small pulmonary arteries had no discrete stenosis and this diffuse hypoplasia might be the result of inadequate blood flow during intrauterine life [Harikrishnan S, Tharakan J, Titus T, Bhat A, Sivasankaran S, Bimal F, Syam Sunder KR, James, KJ. Central pulmonary artery anatomy in right ventricular outflow tract obstructions. Indian Heart Journal 1997;49:624 (Abstract)[18]].