Screening for cardiomyopathy in muscular dystrophy with tissue doppler imaging.

Objective. To study role of Tissue Doppler imaging (TDI) in identification of cardiomyopathy before development of ventricular dysfunction. Methods. Twenty-five patients with Duchenne’s (DMD) and 10 with Becker’s (BMD) muscular dystrophy along with 20 controls were evaluated using TDI. Results. Pulse Tissue Doppler signals of the lateral left ventricle wall (lateral mitral annulus) revealed reduced systolic velocities in the DMD patients (mean ± SD: 7.8 ± 1.1 cm/sec vs. 8.6 ± 1.1 cm/sec in controls) in patients of DMD. 60% of the non ambulatory DMD (ejection fraction 45±9%) patients had mild LV dysfunction and reduced systolic velocities (6.96± 1.7 cm/sec, p<0.05 vs controls). The ambulatory DMD patients also had reduced systolic velocities (7.8+1.1cm/sec ) though ejection fraction was normal. Reduced tissue Doppler systolic velocities in the ambulatory DMD patients with normal conventional echocardiography would suggest that perhaps some of these patients (20% had velocities less than Mean – 2 SD of controls) have early myocardial dysfunction (picked up only as abnormal myocardial velocities). Conclusions. TDI picked up systolic dysfunction of the lateral wall in DMD even when overall LV function was normal conventional echocardiography. Tissue Doppler imaging is a useful technique to pick up early ventricular dysfunction and should be evaluated in larger studies and also with other techniques like magnetic resonance imaging.

Acute hemodynamic effects of sildenafil in patients with idiopathic dilated cardiomyopathy.

Sildenafil, a phosphodiestrase-5 inhibitor, decreases pulmonary artery pressures (PAP) in patients with idiopathic pulmonary hypertension. There is little data pertaining to its use in unselected patients with idiopathic dilated cardiomyopathy (IDCM). A single oral dose of sildenafil (50 mg) was administered to 11 patients (mean age 44.9 +/- 7 years, 7 males) with IDCM with left ventricular ejection fraction < or = 40% in New York Heart Association class II/III at the time of right heart catheterization. There was a significant decrease in pulmonary artery systolic pressure (from 31.5 +/- 9.7 to 19.0 +/- 5.2 mmHg, p < 0.001) and pulmonary vascular resistance (PVR) (from 3.0 +/- 2.1 to 1.6 +/- 0.8 dyne/s/m(2)/cm(5), p = 0.01) following sildenafil administration. The systemic vascular resistance (SVR) and pulmonary wedge capillary pressure also significantly decreased. No significant differences in heart rate, cardiac index and PVR/SVR ratio were observed. There were no side effects documented. Sildenafil produces favorable vasodilation in both pulmonary and systemic vascular beds with decrease in left ventricular filling pressures, in stable patients with IDCM.

Preclinical and early clinical experience with a biodegradable polymer-based, rapamycin-eluting, Indian drug-eluting coronary stent: the BIO-RAPID study.

OBJECTIVE: To evaluate the performance of a biodegradable polymer based rapamycin-eluting coronary stent in a porcine model and demonstrate its safety and efficacy in the treatment of patients with de novo coronary stenosis. BACKGROUND: The indefinite presence of the polymer after the implantation of drug-eluting stents may initiate and sustain inflammation and contribute to the occurrence of late complications. METHODS: Seven study stents and 5 polymer-coated (control) stents were implanted in porcine carotid arteries. Histomorphometric analysis was performed 8 weeks after stent implantation. After establishing the safety of the stent in the animal model, a single-center, non-randomized study in patients with de novo coronary artery lesions was performed. Forty-nine stents were implanted in 43 patients. The 6-month clinical follow-up was 91% (39/43) and angiographic follow-up was 67% (29/43). The primary safety endpoint was the occurrence of 30-day major adverse cardiovascular events (MACE) and the principal efficacy endpoint was the 6-month angiographic late loss and binary restenosis rate. RESULTS: In the porcine model, the study stent showed acceptably low injury, inflammation and fibrin scores. There was a quantitative reduction in neointimal hyperplasia which was not statistically different from the control stent. However, in the first-in-man evaluation, there was significant suppression of intimal growth as evidenced by an angiographic late loss of 0.28 +/- 0.45 mm at 6 months. The restenosis rate was 10.3% (3/297). There was no death, stent thrombosis or myocardial infarction at 30 days or at 6 months. The 6-month target lesion revascularization rate was 3.47 percent; (1/29). CONCLUSION: This preclinical and early clinical experience demonstrates the safety and efficacy of a novel biodegradable polymer-based rapamycin-eluting coronary stent.

Non-invasive diagnosis of coronary artery disease with 16-slice computed tomography.

BACKGROUND: The gold standard for the diagnosis of coronary artery disease (CAD) is catheter angiography. However, catheter angiography is invasive and may not always be followed by interventional therapy. We report our results with the use of multislice computed tomography (MSCT) as a non-invasive diagnostic tool for CAD. METHODS: Thirty-one patients (26 with chronic stable angina, 5 with coronary anomalies) underwent 16-slice MSCT and catheter angiography. Vessels < 1.5 mm in diameter were excluded. The ability of MSCT to detect obstructive CAD (stenosis > or =50% of the diameter) was evaluated in pre-defined vessel segments. The association of calcium score with obstructive CAD, and the effect of heart rate on distal vessel visibility were also studied. RESULTS: There were 29 men and 2 women (age range: 36-80 years; mean [SD]: 53 [11] years). Of the 403 vessel segments, 391 were > 1.5 mm in diameter and 321 were interpretable on both modalities. The non-interpretability rates were 7% (26/391) for catheter angiography and 14% (54/ 391) for MSCT, with distal location (64%; 34), motion artifacts (29%; 16) and calcification (7%; 4) being chiefly responsible in case of the latter technique. The sensitivity, specificity, positive and negative predictive values of MSCT were 85% (95% confidence interval [CI]: 73-93), 94% (95% CI: 90-96), 76% (95% CI: 64-85) and 96% (95% CI: 93-98), respectively. MSCT correctly classified patients with no, single-, double- and triple-vessel disease in 87% of cases. One patient was incorrectly excluded on MSCT; catheter angiography showed 50%-70% stenosis in this case. Patients with obstructive CAD had a higher Agatston score equivalent (p=0.03). There was no significant effect of heart rate on distal segment visibility. MSCT correctly identified all coronary anomalies. CONCLUSION: MSCT has a good potential for the detection of coronary stenosis, and may be most useful for excluding CAD (due to its high negative predictive value). It accurately delineates coronary anomalies.

Screening for pre-clinical hypertrophic cardiomyopathy by tissue Doppler imaging.

BACKGROUND: Hypertrophic cardiomyopathy is an autosomal dominant inherited disorder. On a routine clinical basis, genetic analysis is both time consuming and impractical at present. Thus, use of tissue Doppler imaging as a surrogate for genetic screening is an attractive option. METHODS AND RESULTS: Fifty-five first-degree relatives of 15 patients with hypertrophic cardiomyopathy were screened. Of them, two were found to have hypertrophic cardiomyopathy and were included in Group 1, which hence had 17 patients with overt hypertrophic cardiomyopathy. Group 2 had 53 family members who did not manifest any overt echocardiographic abnormality. Twenty healthy volunteers comprised Group 3. Doppler tissue myocardial longitudinal velocities were measured in systole and early diastole and with atrial contraction at the medial mitral annulus, lateral mitral annulus, mid lateral wall and mid interventricular septum. The tissue Doppler characteristics were analyzed for the presence of abnormalities suggestive of subclinical myocardial involvement. Myocardial velocities were highest in the normal control group and lowest in the hypertrophic cardiomyopathy group. The velocities of the relatives without overt hypertrophy were intermediate in range. Of the 53 relatives screened, nine (17%) subjects showed tissue Doppler abnormality in the systolic and early diastolic velocities at the medial and lateral mitral annulus suggestive of a possibility of pre-clinical hypertrophic cardiomyopathy and a carrier state for a hypertrophic cardiomyopathy. Twenty-two of the 53 screened members had a mean early diastolic velocity less than 13.5 cm/s, among this group 9 had an ejection fraction more than 68%. These findings suggest that at least 16.7% of the screened population may carry beta-myosin heavy chain mutation. CONCLUSIONS: Screening for hypertrophic cardiomyopathy is feasible and tissue Doppler imaging is a sensitive and easy means to detect subclinical myocardial involvement in apparently normal family members without overt hypertrophy.

Familial hypercholesterolemia.

Abnormalities in the lipid profile though uncommon in pediatric practice pose an increased risk for developing heart disease. Studies suggest that adult cardiovascular disease has its roots in children and young adults. A significant correlation between atherosclerotic changes in children and young adults and total and LDL cholesterol levels also exists. The association is particularly true for Familial Hypercholesterolemia. We report a young boy aged 14 years who presented with all the features of Familial Hypercholesterolemia.

Micropulmonary arteriovenous fistula causing cyanosis.

This article presents a young girl who came to the Department of Pediatrics, AIIMS, with cyanosis and was diagnosed to be having multiple pulmonary arteriovenous fistulae. The cardiovascular evaluation was normal and so was the respiratory evaluation. The desaturation did not improve with oxygen. The chest X-ray was also normal. The echocardiogram did not reveal any abnormality but agitated saline contrast echocardiography suggested an extracardiac right to left shunt which was confirmed on pulmonary angiography.