Mitral Stenosis, LV Aneurysm, Myocardial Bridge, and Myocardial Infarction: The Mystery Demystified.

Coronary embolisation, spontaneous coronary artery dissection, and myocardial bridges are rare causes of Myocardial Infarction (MI) in the youth. Here, we report a young male who developed myocardial infarction at the age of 19. Investigations revealed that he had mitral stenosis, myocardial bridge, and angiographic features of healed coronary dissection.

Trans-catheter closure of atrial septal defect: Balloon sizing or no Balloon sizing - single centre experience.

BACKGROUND: Selecting the device size using a sizing balloon could oversize the ostium secundum atrial septal defect (OSASD) with floppy margins and at times may lead to complications. Identifying the firm margins using trans-esophageal echocardiography (TEE) and selecting appropriate-sized device optimizes ASD device closure. This retrospective study was undertaken to document the safety and feasibility of device closure without balloon sizing the defect. METHODS: Sixty-one consecutive patients who underwent trans-catheter closure of OSASD guided by balloon sizing of the defect and intra procedural fluoroscopy (group I) and 67 consecutive patients in whom TEE was used for defect sizing and as intraprocedural imaging during device deployment (group II) were compared. The procedural success rate, device characteristics, and complications were compared between the two groups. RESULTS: The procedure was successful in 79.7 % patients. The success rate in group II (60 of 67, 89.6%) was significantly higher than in group I (41 of 61, 67.2 %) (P = 0.002). Mean upsizing of ASD device was significantly lower in group II (P < 0.001). TEE also provided better success rate with smaller device in subjects with large ASD (>25 mm) and in those who were younger than 14 years of age. There were four cases of device embolization (two in each group); of which one died in group II despite successful surgical retrieval. CONCLUSION: Balloon sizing may not be essential for successful ASD device closure. TEE-guided sizing of ASD and device deployment provides better success rate with relatively smaller sized device.

Long-term functional assessment after correction of tetralogy of Fallot in adulthood.

BACKGROUND: Tetralogy of Fallot presenting in adulthood is a surgical challenge. We present the long-term outcomes of surgical correction in this subset of patients, including results of postoperative effort tolerance as assessed by treadmill testing. METHODS: Fifty-eight patients older than 18 years operated on between January 1995 and June 2004 are included in the study. Mean age at surgery was 22.5 +/- 5 years. Forty-seven patients were in New York Heart Association functional class II and 11 were in class III. Two patients had previous shunts. Forty-four patients received a transannular patch, and 14 had a right ventricular outflow tract patch. The prospective arm objectively assessed postoperative ventricular function by treadmill testing and echocardiography. RESULTS: Hospital mortality was 6.9%. Follow-up was 89% complete, with mean follow-up of 69.9 +/- 43 months. Late mortality occurred in 2 patients, both with infective endocarditis. Significant improvement in functional class was demonstrated (p < 0.001). Eight patients had significant pulmonary regurgitation on follow-up. The probability of survival after repair was 89% at 15 years. Thirty-five of 36 patients who underwent treadmill testing had good effort tolerance, with an average of 10.47 +/- 1.4 metabolic equivalents achieved. None had a positive result. One patient with transannular patch, in functional class III, had fair exercise tolerance with severe pulmonary regurgitation on echocardiography. CONCLUSIONS: Repair of adult tetralogy of Fallot has acceptable morbidity and mortality rates with good long-term surgical outcome in terms of effort tolerance as demonstrated by treadmill testing. Transannular patching does not appear to be a significant risk factor for right ventricular failure at long-term follow-up.

Right ventricular pacing via left superior vena cava.

Negotiating the pacing lead into the right ventricle via left superior vena cava, at times, can be difficult. We report two such cases in which pacing leads were introduced into the right ventricle via left superior vena cava, with the help of stylet tip shaped into a large pigtail loop.

Novel deletions in MYH7 and MYBPC3 identified in Indian families with familial hypertrophic cardiomyopathy.

Mutations causing familial hypertrophic cardiomyopathy (HCM) have been described in at least 11 genes encoding cardiac sarcomeric proteins. In this study, three previously unknown deletions have been identified in the human cardiac genes coding for beta-myosin heavy chain (MYH7 on chromosome 14) and myosin-binding protein-C (MYBPC3 on chromosome 11). In family MM, a 3-bp deletion in MYH7 was detected to be associated with loss of glutamic acid in position 927 (DeltaE927) of the myosin rod. In two other families (HH and NP, related by a common founder) a 2-bp loss in codon 453 (exon 16) of MYBPC3 was identified as the presumable cause of a translation reading frame shift. Taken together 15 living mutation carriers were investigated. Six deceased family members (with five cases of premature sudden cardiac death (SCD) in families MM and NP) were either obligate or suspected mutation carriers. In addition to these mutations a 25-bp deletion in intron 32 of MYBPC3 was identified in family MM (five carriers) and in a fourth family (MiR, one HCM patient, three deletion carriers). In agreement with the loss of the regular splicing branch point in the altered intron 32, a splicing deficiency was observed in an exon trapping experiment using MYBPC3 exon 33 as a test substrate. Varying disease profiles assessed using standard clinical, ECG and echocardiographic procedures in conjunction with mutation analysis led to the following conclusions: (1) In family MM the DeltaE927 deletion in MYH7 was assumed to be associated with complete penetrance. Two cases of reported SCD might have been related to this mutation. (2) The two families, HH and NP, distantly related by a common founder, and both suffering from a 2-bp deletion in exon 16 of MYBPC3 differed in their average phenotypes. In family NP, four cases of cardiac death were documented, whereas no cardiac-related death was reported from family HH. These results support the notion that mutations in HCM genes may directly determine disease penetrance and severity; however, a contribution of additional, unidentified factors (genes) to the HCM phenotype can-at least in some cases-not be excluded. (3) The deletion in intron 32 of MYBPC3 was seen in two families, but in both its relation to disease was not unequivocal. In addition, this deletion was observed in 16 of 229 unrelated healthy individuals of the population of the South Indian states of Kerala and Tamil Nadu. It was not seen in 270 Caucasians from Russia and western Europe. Hence, it is considered to represent a regional genetic polymorphism restricted to southern India. The association of the deletion with altered splicing in transfected cells suggests that this deletion may create a "modifying gene", which is per se not or only rarely causing HCM, but which may enhance the phenotype of a mutation responsible for disease.

Percutaneous coil closure of recanalised anomalous origin of left coronary artery from pulmonary artery.

An asymptomatic boy underwent surgical correction of anomalous origin of left coronary artery from pulmonary artery (ALCAPA) with trans-pulmonary artery interruption and saphenous vein grafting to left anterior descending coronary artery. He developed a shunt through the re-canalised pulmonary artery end of the ALCAPA which was successfully embolised using a detachable PDA coil delivered into the left main coronary artery from the pulmonary artery.