Percutaneous treatment of Lutembacher syndrome in a case with difficult mitral valve crossing.

Most cases of combination congenital cardiac anomalies are treated with open-heart surgeries because the coexisting anomalies change the cardiac anatomy in an adverse way, making catheter manipulations complex. Lutembacher syndrome is a combination of acquired mitral stenosis and congenital ostium secundum atrial septal defect. The large defect in the septum makes an Inoue balloon catheter unstable, which provides excessive space for free floatation of the catheter, making its passage into the left ventricle difficult by Inoue technique. We present a case of elective definitive percutaneous treatment of Lutembacher syndrome, discussing the technical difficulties faced in mitral valve crossing and reviewing the possible strategies to improve chances of success.

Percutaneous transvenous mitral commissurotomy for significant calcific mitral stenosis: utility of the stepwise balloon dilatation technique and follow-up results.

The present study examined the utility of the stepwise balloon dilatation technique in 41 patients with significant calcific mitral stenosis undergoing percutaneous transvenous mitral commissurotomy (PTMC). Thirty-five patients (85.4%) had a successful procedure; one patient developed cardiac tamponade and underwent mitral valve replacement. The mitral valve area increased from 0.9 +/- 0.2 cm2 to 1.7 +/- 0.3 cm2 following PTMC. Increase in mitral regurgitation (MR) was seen in 11 patients (26.8%). All patients showed improvement in functional class of > or =1 level following PTMC, which was sustained in 34 patients at follow-up. At a mean follow-up period of 20 +/- 12 months (range 3-51 months) in 35 patients, 26 patients (74.3%) were in New York Heart Association (NYHA) functional Class I, 8 patients (22.9%) were in NYHA Class II, and 1 patient (2.8%) was in NYHA Class III. The cumulative 4-year cardiac event-free survival rate was 81.8%. However, patients with grade 4+ calcification had only 50% event-free survival rate. At follow-up, an increased incidence of cardiac events was seen in female patients as compared with male patients (83.3% versus 16.7%). Restenosis was seen in 3 patients (8.6%). One patient underwent repeat PTMC 37 months after the initial procedure. There was no incidence of death or mitral valve replacement at follow-up. We conclude that the stepwise balloon dilatation technique can be safely and effectively applied for patients with significant calcific mitral stenosis to achieve an optimal mitral valve area with low incidence of significant increase in MR. Favorable long-term benefits also accrue in the form of improved functional status and low incidence of repeat procedures (repeat PTMC or mitral valve replacement). The majority of patients (74.3%) were in NYHA functional class I without medication. Patients with grade 4+ calcification show less benefit from PTMC and may be considered for mitral valve replacement. Cardiac events occur more frequently in female patients than in male patients during follow-up.

Tetralogy of Fallot in adults--107 cases.

Clinical and haemodynamic profile of 107 adult patients above the age of 15 years with TOF was analysed. Cardiac catherization and selective cine-angiography were performed in all cases. Infundibular pulmonary stenosis, mal-alignment type of ventricular septal defect, mitral-aortic fibrous continuity and equal systolic pressures in both the ventricles and aorta were considered mandatory for the diagnosis of Tetralogy of Fallot. Aortic regurgitation was seen in 26 cases (24%), tricuspid regurgitation in 22 cases (21%), absent pulmonary valve in 3 cases (3%), branch pulmonary artery stenosis in 9 case (8.4%), major aortopulmonary collaterals in 15 cases (14%), right atrial pressure was more than 10 mmHg in 10 cases (11%) and right ventricular end diastolic pressure more than 9 mmHg in 73 cases (68%). The left ventricular end diastolic pressure was above 13 mmHg in 58 cases (54%).

Ventricular septal defect with aortic regurgitation: a hemodynamic and angiographic profile in Indian subjects.

The records of 362 patients of Ventricular Septal Defect (VSD) were analysed to find out the incidence of aortic regurgitation (AR) and their hemodynamic and angiographic features. Thirty-seven patients (10.2%) were found to have AR, whose mean age was 13.4 years (range: 2-45) and male to female ratio was 5:1. Of the 37 cases 31 (84%) had infracristal and 6 (16%) had supracristal VSD. In 31 patients with infracristal VSD the prolapsing cusp was Right Coronary Cusp (RCC) in 14 (48%), Noncoronary Cusp (NCC) in 12 (41%) and both RCC and NCC in 3 (11%). Of the 6 patients with supracristal VSD the prolapsing cusp was RCC in 5 (83%) and NCC in 1 (17%). In two patients the AR was due to bicuspid aortic valve. The pulmonary artery pressure was normal in 26 of 37 (70.2%) patients and the left to right shunt was 1.5:1 or less in 23 of 37 (62%) patients. Nineteen of the 37 patients (51.3%) had grade I or II AR and the remaining 18 (48.7%) had grade III or IV AR. There was no relationship between the severity of AR and the location of the VSD. In conclusion, in this series, the incidence of VSD+AR is relatively higher and that of supracristal VSD is lower. In majority of patients the left to right shunt is small and pulmonary artery pressure within normal limits. The prolapse of RCC is more common in supracristal VSD and there is no relation between the severity of AR and the location of the VSD.

Tetralogy of Fallot with hypertrophic cardiomyopathy--an unknown combination.

A hitherto unknown association of Tetralogy of Fallot (TF) and Hypertrophic Cardiomyopathy (HCM) diagnosed by two dimensional echocardiography, cardiac catheterization and angiocardiography is reported. Patient underwent emergency aortopulmonary shunt successfully. The literature is reviewed in brief.

Myocardial bridge (MB): an angiographic curiosity?

The coronary angiograms of 1,500 cases performed between 1981 and 1989 were analysed to find out the incidence of Myocardial Bridge (MB) and its significance as regards myocardial ischemia. Sixteen of these (1.06%) were found to have MB. Their ages ranged from 27-70 years (m = 49.2) and male:female ratio was 13:3. Out of 16 patients, 7 (group A) had associated coronary artery disease (CAD) (7 of 1421; 0.49%) and remaining 9 (group B) had no associated CAD (9 of 79; 11.39%). All the MB were found on left anterior descending artery (LAD) (3 on proximal LAD and 13 on mid LAD). No MB was found on right coronary artery (RCA) or circumflex arteries. The location of the MB did not affect the pattern of CAD. Chronic stable angina was the commonest presenting symptom in group A patients (5 out of 7) and atypical angina in group B patients (5 out of 9). Majority of group B patients had either normal or nonspecific ST-T changes in ECG (7 out of 9). However, the presence of previous myocardial infarction or ECG evidence of 'Q' wave infarction (2 out of 2) was always associated with significant CAD. Similarly, regional wall motion abnormalities on echocardiogram were always found in patients with significant CAD and old myocardial infarction. All 9 patients with MB and normal coronary arteries were managed conservatively with good relief of symptoms, whereas other seven patients were managed on the merits of the underlying CAD. In conclusion, the MB is a normal variant found incidentally on coronary angiography, and does not have any definite clinical correlations or pathological significance.

Detection of silent myocardial ischemia by Holter monitoring and its relation to severity of coronary artery disease.

Thirty-five patients of chronic stable angina, unstable angina and post MI angina, who were on medical treatment, underwent 24 hours Holter monitoring and coronary angiography to find out the incidence of Silent Myocardial Ischemia (SMI) and its relation to anatomic severity of coronary artery disease. Total duration of Holter monitoring was 835.32 hours (average 23.40 hours per patient) with 48 ischemic episodes out of which 16 were painful and 32 painless. Total duration of painful episodes was 189 minutes and that of painless episodes was 428 minutes (70% was constituted by SMI). Out of 35 patients, 6 (17.14%) had SMI; 2 of 17 (11.7%) of chronic stable angina, 2 of 8 (25%) of unstable angina, and 2 of 10 (20%) of post-infarction angina patients. On analysis of coronary angiogram, all 6 (100%) patients with SMI, and only 22 out of 29 (76%) without SMI, had severe multiple coronary artery disease. Thus, although the overall incidence of SMI in this series is low, its presence invariably indicates a severe degree of coronary artery disease.

Severe syphilitic aortic regurgitation with bilateral critical coronary ostial stenosis.

A forty-year-old male with syphilitic severe aortic regurgitation and critical bilateral coronary ostial stenosis, proved by cardiac catheterization and angiocardiography, is presented. He underwent successful aortic valve replacement and coronary artery bypass grafting with gratifying results.