Giant right atrium due to congenital dysplastic tricuspid valve in an elderly female patient.

Isolated tricuspid valve disease is rare. We report the case of a 65-year-old female patient who presented with right heart failure. Initial echocardiographic evaluation revealed a giant right atrium (RA) with severe tricuspid inflow obstruction with regurgitation. A comprehensive two- and three-dimensional echocardiographic evaluation revealed a dysplastic tricuspid valve resulting in the inflow obstruction and regurgitation. Rheumatic and carcinoid etiologies were ruled out by relevant tests. It is very rare for dysplastic tricuspid valve to present in late adulthood. The classic echocardiographic findings are discussed followed by a brief review of the literature.

Total anomalous systemic venous drainage in left heterotaxy syndrome.

Total anomalous systemic venous drainage is an extremely rare congenital heart defect. In this study we describe an 11-year-old girl who presented with a history of fatigue and central cyanosis that she had had since early childhood with unremarkable precordial examination results. Investigations revealed left heterotaxy with all systemic venous drainage to the left-sided atrium with non-compaction of the left ventricle.

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 patient's 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 patient's 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.

Prospective study of infective endocarditis from a developing country.

BACKGROUND: There has been a considerable change in the profile of infective endocarditis (IE) worldwide. However, prospective studies of IE from developing countries are rare. AIM: The aim of this study was to evaluate predisposing factors, clinical presentation, echocardiography, microbiology, in-hospital course, and outcomes of patients with definite IE. METHODS: This was a prospective observational study of consecutive cases of definite IE as per the modified Duke criteria. RESULTS: Between 2004 and 2006, a total of 104 cases of definite IE were identified. The mean age was 23.5 years (interquartile range, 9-38 years), with a male preponderance (2.5:1). Congenital heart disease accounted for 39.4% cases of IE, followed by native valve disease (29.8%), prosthetic valve disease (20%), and normal valves (7.7%). Echocardiography was diagnostic in 96.7% of the patients. Positive cultures were obtained in only 41% of the cases, with staphylococci and streptococci being the most common organisms. Seventy-three percent of the cases had at least 1 major complication. Only 15% of the cases underwent surgery for IE. The in-hospital mortality was 26%. On multivariate analysis, an underlying heart disease other than native valve disease and septic shock were independent predictors of mortality. CONCLUSION: The mean age of IE in India is significantly lower than that in the West. Unlike previous reports, congenital heart disease is the major predisposing factor. Culture positivity rates and surgery for IE are unacceptably low. In conclusion, IE in India is associated with a very high morbidity and mortality.

Timing of adverse events during fibrinolytic therapy with streptokinase for left-sided prosthetic valve thrombosis.

Serious bleeding and embolic events are common after fibrinolytic therapy (FT) for left-sided prosthetic valve thrombosis (PVT), and are believed to occur more frequently with prolonged fibrinolytic infusions. But there is no data from prospective studies examining this contention. Our aim was to determine the timing of adverse events after FT with streptokinase (SK) for left-sided PVT. In a post-hoc analysis involving all 120 participants from a randomized controlled trial of FT for left-sided PVT, we determined the timing of occurrence of a composite of death, major bleeding, embolic stroke, or non-central nervous system systemic embolism, in relation to the duration of SK infusion. The composite outcome occurred in 20 (16.7%) of the 120 patients. Adverse events were more frequent within 12 h of initiating treatment than later (28% vs. 10%; OR 3.75, 95% CI 1.25-11.20, P = 0.018). Contrary to current thinking, adverse events can occur early during fibrinolytic therapy for left-sided PVT. This has important implications for the use of fibrinolysis as first line treatment for left-sided PVT.

Accelerated infusion of streptokinase for the treatment of left-sided prosthetic valve thrombosis: a randomized controlled trial.

BACKGROUND: No large prospective studies have evaluated the efficacy of fibrinolytic therapy for left-sided prosthetic valve thrombosis, yet it remains the first line of treatment in developing countries. METHODS AND RESULTS: We performed a randomized controlled trial comparing an accelerated infusion with the conventional infusion of streptokinase in 120 patients with a first episode of left-sided prosthetic valve thrombosis. The primary outcome measure was the occurrence of a complete clinical response, defined as objectively documented complete restoration of valve function in the absence of major complications. The secondary outcome was a composite of death, major bleeding, embolic stroke, or non-central nervous system systemic embolism. Patients were recruited over a 2.5-year period at a single center in India. Complete clinical response occurred in 38 (64.4%) of 59 patients with the accelerated infusion compared with 32 (53.3%) of 60 with the conventional infusion (hazard ratio 1.6, 95% confidence interval 0.9 to 2.5, P=0.055). There was no significant difference in the occurrence of the composite secondary outcome (hazard ratio 1.4, 95% confidence interval 0.5 to 3.5, P=0.50) or major bleeding (hazard ratio 2.2, 95% confidence interval 0.6 to 7.7, P=0.24) with the accelerated infusion. The success rate with fibrinolytic therapy was low overall (59%) and very low in patients in New York Heart Association functional class III/IV (24%). CONCLUSIONS: The large number of patients recruited from a single center underscores the massive burden of prosthetic valve thrombosis in developing countries. Fibrinolytic therapy with streptokinase is less efficacious than previously believed. The accelerated streptokinase infusion is not better than the standard infusion for left-sided prosthetic valve thrombosis. Developing countries urgently need more effective strategies to prevent and treat prosthetic valve thrombosis.

Anomalous origin of the left coronary artery from the pulmonary artery (ALCAPA) presenting as a complete heart block.

A 51-year-old previously asymptomatic man presented with complete heart block (CHB). During pacemaker implantation, fluoroscopy showed a peculiar pattern of cardiac calcification. Coronary angiography, performed to determine the origin of calcification, demonstrated an anomalous origin of the left coronary artery from the pulmonary artery (ALCAPA). A left ventriculogram showed normal ventricular contraction. Echocardiography demonstrated normal systolic function without any regional wall motion abnormality. The endocardium of the mid and basal portions of the anteroseptal, anterior and anterolateral walls as well as both of the papillary muscles were calcified. Specifically noted was a calcific bar extending across the base of the interventricular septum (IVS) on both the echocardiogram and the left ventricle angiogram. The development of CHB in the absence of transmural myocardial infarction is intriguing. It is likely that endocardial fibroelastosis during infancy led to endocardial fibrosis and scarring subsequent calcium deposition. Extension of this calcification into the conduction system may have led to CHB. This is the first report of an adult patient with ALCAPA presenting with CHB.

Thrombolysis with reteplase in acute pulmonary embolism.

OBJECTIVE: Reteplase (recombinant plasminogen activator) is a mutant of alteplase. It has a longer half-life than its parent molecule and has shown better vessel patency rates in acute myocardial infarction. In this study, we analyzed the efficacy and safety of reteplase in acute pulmonary embolism (PE). METHODS: This observational study included patients with high- and intermediate-risk acute PE, presenting within 14 days of symptom onset. The patients were treated with reteplase, which was given in two bolus doses of 10 U each, 30 min apart, along with intravenous heparin. Patients with hemodynamic compromise (high-risk or massive PE) and normotensive patients with evidence of right ventricular (RV) dysfunction (intermediate-risk or submassive PE) on echocardiography or computed tomography were included in the study. The efficacy outcomes assessed were in-hospital death and improvement of RV function by echocardiography. The safety outcomes were major bleeding, minor bleeding, and ischemic or hemorrhagic stroke during hospitalization. RESULTS: Of the 40 patients included, 25% were classified as high risk with hemodynamic compromise and 75% were classified as intermediate risk. RV dysfunction was present in all the patients (100%). Concomitant lower extremity deep vein thrombosis was present in 55% of the patients. The mortality rate was 5%. There was significant improvement in RV function and reduction in pulmonary artery systolic pressure and tricuspid regurgitation severity. There was no major bleeding event or stroke, and 7.5% patients had minor extracranial bleeding. CONCLUSIONS: Double-bolus reteplase given with heparin is effective in the treatment of high- and intermediate-risk PE, with minimal risk of bleeding.

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.

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.

Transjugular percutaneous transvenous mitral commissurotomy (PTMC) using conventional PTMC equipment in rheumatic mitral stenosis with interruption of inferior vena cava.

BACKGROUND: Isolated interruption of the inferior vena cava (IVC) is a rare anomaly. We report a series of 4 cases of isolated interruption of the IVC that coexisted with rheumatic mitral stenosis. Interrupted IVC precludes the use of the femoral approach to percutaneous transseptal mitral commissurotomy (PTMC). We describe the jugular approach to PTMC in such cases using conventional PTMC equipment. METHODS AND RESULTS: The mean pre-PTMC mitral valve area was 0.85 cm². Septal puncture was done through the right internal jugular vein with a pediatric Brokenborough needle (Medtronic) using the levophase of pulmonary artery angiogram and the pigtail as guide. The mitral valve was crossed successfully in all cases and appropriately sized Accura balloons (Vascular Concepts) were used for incremental dilatations. Successful balloon dilatation was achieved in all 4 cases (mean post-PTMC mitral valve area of 1.85 cm²) with no complications. CONCLUSION: The jugular approach appears to be a safe and effective alternative in cases of rheumatic mitral stenosis with IVC anomalies, thereby preventing an otherwise necessary surgery.

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.

Superior vena caval stenting for idiopathic mediastinal fibrosis.

Relief of superior vena caval (SVC) syndrome due to non-neoplastic mediastinal disease is a challenge. Surgically created bypass grafts have poor long term patency with significant procedural morbidity. We report a case of SVC syndrome, caused by Idiopathic mediastinal fibrosis, treated percutaneously by balloon angioplasty with nitinol self expanding stent.

An unusual case of dissecting aneurysms involving both coronary sinuses of valsalva.

A 16-year-old boy with a progressive history of dyspnea and palpitations was diagnosed to have rupture of the right and left coronary sinuses on transthoracic echocardiography. The right coronary sinus had ruptured and dissected into the interventricular septum from its base up to the left ventricular apex and all around the lateral wall of the left ventricle up to the base of the papillary muscles. The left coronary sinus was also ruptured, and the rupture was contained by a false aneurysm that dissected into the roof of the left atrium from the aortomitral intervalvular fibrosa. The involvement of multiple coronary sinuses is extremely rare.

Catheter-induced left main coronary artery spasm.

A dramatic illustration of catheter-induced left main coronary artery spasm is described. Timely recognition of spasm and use of intracoronary nitroglycerine confirmed this condition and saved a young woman from an unnecessary coronary artery bypass graft operation.

An uncommon complication after a common procedure.

A 50-year-old female developed cortical blindness 5 hours after coronary angiography. A non-contrast-enhanced computed tomographic scan of the brain revealed bilateral occipital and middle cerebral/posterior cerebral artery watershed territory infarcts with a possibility of subarachnoid hemorrhage (SAH). Magnetic resonance imaging of the brain revealed the above infarcts, but not SAH. The selective vulnerability of occipital lobes to contrast agent toxicity was postulated as the underlying mechanism. The patient had partial recovery of vision.