Ravindranath K. Shankarappa

Acute severe mitral regurgitation following balloon mitral valvotomy: Echocardiographic features, operative findings, and outcome in 50 surgical cases

To analyze the echocardiographic and operative findings with respect to mitral valve anatomy in individuals undergoing emergency surgery for acute severe mitral regurgitation (MR) following balloon mitral valvotomy (BMV). In addition, the clinical profile and outcomes are highlighted.

Accelerated coronary atherosclerosis and H syndrome.

A 12-year-old boy with insulin dependent diabetes mellitus, presented with acute myocardial infarction. Intracoronary thrombolysis with urokinase restored TIMI III flow in the culprit vessel. After stabilisation with medical therapy, unusual clinical findings in the form of cutaneous hyperpigmentation and hypertrichosis, affecting the lower extremities, were appreciated. These and other phenotypic features were consistent with H syndrome, a recently described autosomal recessive genodermatosis, and confirmed by mutation analysis. Despite being on optimal medical therapy for coronary artery disease, the patient presented 3 months thereafter, with unstable angina which was successfully managed with percutaneous coronary intervention. An unusual occurrence of coronary artery disease with accelerated atherosclerosis in a child with H syndrome is presented herein. Identification of further patients with this novel disorder will clarify the possible association, suggested here, with increased risk for coronary or other vascular events.

Free floating right atrial thrombus with massive pulmonary embolism:near catastrophic course following thrombolytic therapy

A 28-year-old policeman presented with left lower limb deep vein thrombus, pulmonary embolism and a highly mobile right atrial clot. Thrombolytic therapy with IV Tenecteplase was administered. Within a few minutes after the Tenecteplase bolus, the patients condition worsened dramatically with severe hypotension and hypoxemia. Immediate bedside transthoracic echocardiogram revealed that the mobile right atrium clot had disappeared completely presumably having migrated to the pulmonary circulation thus worsening the clinical condition. With intensive supportive measures the patients condition was stabilized and he made a complete recovery. Prior to discharge, the echocardiogram revealed normal right ventricular function and a CT pulmonary angiogram performed after 2 months revealed near complete resolution of pulmonary thrombi. Thrombolytic therapy for right heart thrombus with pulmonary embolism can be a reasonable first line therapy but may be associated with hemodynamic worsening due to clot migration.

Infective endocarditis: a rare organism in an uncommon setting.

Facklamia hominis is a rare causative organism of infective endocarditis (IE). Only few cases of infection due to F. hominis have been reported in the literature. We describe a case of IE due to Gram-positive, alpha-haemolytic, catalase-negative coccus F. hominis in an adult patient with rheumatic mitral stenosis. Isolated mitral stenosis is an uncommon valve lesion predisposing to IE. The following paper is being presented to emphasize the possibility of IE due to F. hominis, and laboratories need to be alert of the potential significance in appropriate clinical setting.

Spontaneous asymptomatic coronary artery dissection in a young man

Spontaneous coronary artery dissection (SCAD) is a rare condition that most often presents as acute coronary syndrome or sudden cardiac death. Here we present the case of a young man of 25 years, who had remained asymptomatic in spite of SCAD. This case highlights the fact that spontaneous dissections can occur at a young age and can involve more than one coronary artery, without producing clinical symptoms.

Spectrum of cyanotic congenital heart disease diagnosed by echocardiographic evaluation in patients attending paediatric cardiology clinic of a tertiary cardiac care centre.

BACKGROUND Cyanotic CHD comprises up to 25% of cases of all causes of CHD. RATIONALE There is lack of data about the present spectrum of congenital cyanotic heart disease in the paediatric age group. OBJECTIVE The present study was undertaken to determine the spectrum of patients with congenital cyanotic heart disease in the paediatric age group in tertiary paediatric cardiac care clinic. DESIGN Prospective observational study. SETTING Paediatric cardiac clinic of a tertiary cardiac care centre. METHODS All children aged 0-18 years with suspected cyanotic CHD were provisionally included in this study. They underwent a thorough echocardiographic evaluation, and those patients who had definitive diagnosis of congenital cyanotic heart disease were included for final analysis. RESULTS A total of 119 children met the inclusion criteria. Tetralogy of Fallot and its variant were the most common congenital cyanotic heart disease with proportion of about 44%. Other common malformations were double outlet right ventricle (14%), pulmonary atresia with ventricular septal defect (8%), total anomalous pulmonary venous connection (7%), d-transposition of the great arteries (9%), tricuspid valve anomalies--tricuspid atresia and Ebsteins anomaly--hypoplastic left-heart syndrome, truncus arteriosus, and complex CHD such as single ventricle. CONCLUSION Tetralogy of Fallot and its variants were the most common cyanotic heart disease diagnosed in our patients. As there were a significant proportion of cases with complex cyanotic CHD, paediatric cardiologists should be familiar with the diagnosis and management of all these complex congenital malformations of the heart.

Percutaneous coronary intervention in patients with anomalous origin of coronary artery presenting with acute coronary syndrome: A case series

Coronary artery anomalies are found in 0.6%-1.5% of patients undergoing diagnostic coronary angiogram. Intervention in these patients poses a particular technical challenge secondary to the aberrancies in the vessel origin and course. From March 2011 to February 2013, 13 cases with complex coronary artery anomalies were observed among 2482 patients undergoing CAG (0.52%) at our cath lab. Only three patients had severe stenosis in the anomalous artery sufficient to require an intervention and had presented with myocardial infarction. PCI was performed successfully in these 3 patients two of which had anomalous left circumflex artery and the other having an anomalous right coronary artery.

The challenge produced by familial homozygous hypercholesterolemia when treating premature coronary arterial disease in the young.

BACKGROUND Familial hypercholesterolemia is a monogenic, autosomal dominant disorder caused by mutations in the LDL receptor gene. Familial homozygous hypercholesterolemia results when both the alleles have the defective mutation. It is characterized by cutaneous and tendinous xanthomas, premature corneal arcing, and is associated with an increased risk of coronary arterial disease. It is also seriously underdiagnosed, resulting in delayed treatment. METHODS We present a cross-sectional study of 5 patients with familial homozygous hypercholesterolemia who presented to the department of cardiology at Sri Jayadeva Institute of Cardiology, Bangalore, India. All of them underwent coronary angiography as part of the investigation of their angina. RESULTS All 5 patients were in 2nd or 3rd decade of life, 4 being male, and 4 presenting with effort angina, the other having unstable angina. All had multiple tendinous xanthomas. The majority had significant high grade coronary arterial stenosis. Coronary arterial bypass grafting was necessary in 3, with the others undergoing percutaneous insertion of coronary arterial stents. CONCLUSION Familial homozygous hypercholesterolemia is a potentially dangerous risk factor that can result in premature coronary arterial disease in children and young adults. This can result in severe morbidity and premature death in young individuals. We also emphasise the need to screen first-degree relatives and extended family members, this playing an important role in early detection and treatment. Despite recent advances in treatment using lipid lowering agents, the disease remains a significant challenge.

Isolated Left Ventricular Noncompaction Mimicking Ventricular Mass

Isolated left ventricular noncompaction is an inherited cardiomyopathy characterized by multiple prominent trabeculations with deep intertrabecular recesses. The diagnosis is often missed because echocardiography poses inherent problems of poor echo window in assessment of the LV apex, which is most commonly involved in noncompaction. We report a case in which conventional 2D echocardiography failed to demonstrate multiple prominent trabeculations. Contrast echocardiography confirmed the presence of multiple trabeculations with deep intertrabecular recesses. This report emphasizes the importance of contrast echocardiography in the diagnosis of ventricular noncompaction. (Echocardiography 2011;28:E137‐E139)

Multiple Fractures With Pseudoaneurysm Formation in a Subclavian Artery Stent

A 50-year-old right-handed man (ex-smoker) with a history of left upper limb claudication for the previous 6 years underwent successful left subclavian artery (LSA) angioplasty with stent implantation (8×59 Genesis Stent [Cordis, Warren, NJ]) for 99% ostial and proximal stenosis of the LSA with use of a combined anterograde and retrograde approach (Figure 1A through 1D). The erythrocyte sedimentation rate and the C-reactive protein were normal. No other vascular system was affected. After an asymptomatic period of 4 months, the patient presented with a history of high-grade fever for 7 days, pain and swelling of the left hand, bluish discoloration of finger tips and palms, and restriction of movements of the fingers of left hand (Figure 2A). All left upper limb pulses were well felt. A Doppler arterial study of the left upper limb was also normal. At the diagnostic angiogram, fluoroscopy revealed that the LSA stent had transected at multiple levels (4 levels) (Figure 3A and Movie I of the online-only Data Supplement). The angiogram revealed the presence of 2 pseudoaneurysms, one at the origin …