Giant aneurysm of the vertical vein in a case of supracardiac total anomalous pulmonary venous connection.

A 17-year-old female presented with mild cyanosis. Imaging showed supracardiac total anomalous pulmonary venous connections with a vertical vein travelling between the left pulmonary artery and the left bronchus with significant obstruction. There was a huge post-stenotic aneurysm of the vertical vein.

A classical case of the Gasul phenomenon.

This case demonstrates the development of secondary infundibular stenosis in a 10-year-old male child with documented large non-restrictive perimembranous ventricular septal defect in infancy - the classical Gasul phenomenon.

Classical Cardiovascular Manifestations of Marfan Syndrome.

Presence of multiple cardiovascular manifestations of the Marfan syndrome in the same patient is not commonly encountered. We present a 49 year-old lady with this syndrome who presented with decompensated heart failure. Evaluation revealed presence of extensive Stanford type A aortic dissection alongwith severe aortic and mitral incompetence. However, the patient declined surgery and was discharged on medical management. At a year's follow-up, she had dyspnea of NYHA class II with persistent cardiovascular findings.

Supracardiac total anomalous pulmonary venous connection with bilateral (right and left) vertical veins and bilateral obstruction.

This report describes a rare form of obstructed supracardiac total anomalous pulmonary venous connection with two vertical veins (right and left) draining the right- and the left-sided pulmonary veins respectively without formation of a common chamber and with bilateral obstruction. Surgery for these patients is technically challenging due to the absence of a common chamber, and the prognosis is worse than for patients with a common chamber. Also, it is important to be aware of this entity so that an accurate noninvasive preoperative diagnosis by echocardiography alone without invasive cardiac catheterization is possible. This facilitates emergency surgical repair without delay, which is crucial to improvement of the outcome.

Acute pancreatitis complicated by ST-elevation myocardial infarction.

BACKGROUND: Electrocardiographic abnormalities mimicking myocardial ischemia have been reported in intra-abdominal conditions, including acute pancreatitis. However, the occurrence of ST-elevation myocardial infarction (STEMI) is rare. OBJECTIVES: To present a case report of a young man with acute pancreatitis subsequently complicated by acute STEMI. The diagnosis and management of STEMI in acute pancreatitis can present unique diagnostic and therapeutic challenges, which are reviewed. CASE REPORT: A 31-year-old man with no conventional coronary risk factors presented with acute abdominal pain, elevated pancreatic enzymes, and computed tomography scan findings of acute pancreatitis. The patient developed chest discomfort and presented to us on Day 2 with electrocardiographic evidence of an evolved extensive anterior wall myocardial infarction. Cardiac troponin I levels were elevated, and the electrocardiogram showed regional wall motion abnormalities in the left anterior descending territory (LAD). Coronary angiography done after stabilization showed a thrombus in the LAD, with no atherosclerotic lesions whatsoever. Hemostatic abnormalities are known in acute pancreatitis, and the development of a transient hypercoagulable state may be responsible for thrombotic complications. The overlap of some of the symptoms of the two conditions may cause diagnostic difficulty. Management issues include the choice of revascularization therapy, the safety of antiplatelet and anticoagulant therapy, intravenous fluid administration, and the use of cardiac medications that potentially can cause hypotension. CONCLUSION: The diagnosis and management of STEMI in the setting of acute pancreatitis can be challenging. In the absence of guidelines, a multidisciplinary approach individualized to the patient's clinical situation may be most appropriate.

Unilateral Breast Uptake of 99mTc-MIBI in a Lactating Woman During Myocardial Perfusion Imaging.

ABSTRACT: A 34-year-old woman, who was 11 months postpartum, underwent 99mTc-MIBI myocardial perfusion SPECT imaging for atypical symptomatology with normal baseline electrocardiogram and 2-dimensional echocardiography. She was lactating on and off, preferentially from the right breast. Analysis of the raw images revealed unilateral intense tracer uptake in the right breast region that persisted in the delayed spot views (24-hour postinjection). Although bilateral breast uptake of 99mTc-MIBI may be seen in postpartum scenario, unilateral breast uptake can also occur in patients with preferential lactation from 1 breast as seen in this case and should not be mistaken for pathology.

Brothers with constrictive pericarditis - A novel mutation in a rare disease.

Familial constrictive pericarditis is extremely rare. We report a case of two brothers both suffering constrictive pericarditis along with having multiple painless joint deformities. Genetic workup confirmed the clinical diagnosis of camptodactyly-arthropathy-coxa vara-pericarditis (CACP) syndrome CACP syndrome and also revealed a rare mutation in the causative gene.

Echocardiographic parameters in clinical responders to surgical pericardiectomy - A single center experience with chronic constrictive pericarditis.

BACKGROUND: Chronic constrictive pericarditis (CCP) is the end result of chronic inflammation of the pericardium. Developing countries continue to face a significant burden of CCP secondary to tuberculous pericarditis. Surgical pericardiectomy offers potential cure. However, there is paucity of echocardiography data in post-pericardiectomy patients vis-a-vis their clinical status. We studied the changes in multiple echocardiographic parameters in these patients before and after pericardiectomy. METHODS: Twenty-three patients (14 men, 9 women) who underwent pericardiectomy for CCP in the last 5 years (from January 2009 to December 2014) were subjected to detailed clinical and echocardiographic evaluation during the study period (between June 2013 and December 2014). Patients with residual symptoms of NYHA class II and below were considered as 'responders'. The data thus obtained were compared to the pre-operative parameters. RESULTS: After pericardiectomy, the incidence of vena caval congestion decreased from 100% to 15% (p<0.001). There was significant reduction in the mean left atrial size from 39.33±10.52mm to 34.45±10.08mm (p<0.001) and also the ratio of left atrium to aortic annulus from 1.93 to 1.69 (p<0.001) among 'responders' to pericardiectomy. Septal bounce was observed to persist in 5 (25%) patients after pericardiectomy. There was significant respiratory variation of 39.23±15.11% in the mitral E velocity before pericardiectomy. After pericardiectomy, this variation reduced to 14.43±7.76% (p<0.001). There was also significant reduction in the respiratory variation in tricuspid E velocities from 31.33±18.81% to 17.35±16.26% (p<0.001). After pericardiectomy, the mean ratio of mitral annular velocities, medial e': lateral e', reduced from 1.08 to 0.87 (p<0.03). The phenomenon of 'annulus reversus' was found to persist in 6 'responders', thereby reflecting a 50% reduction in its incidence after pericardiectomy (p<0.001). The ratio of mitral E to medial e' (E/e') increased from 4.21±1.35 before pericardiectomy to 6.91±2.62 after pericardiectomy (p=0.001). CONCLUSION: Among clinical responders to surgical pericardiectomy, echocardiographic assessment revealed a significant reduction in vena caval congestion, LA size, ratio of LA to aortic annulus, septal bounce, respiratory variation in mitral and tricuspid E velocities, mitral annular medial e' and the phenomenon of annulus reversus. Also, there was a significant rise in minimum tricuspid and mitral E velocities and the E/e' ratio.

Simultaneous transcatheter closure of ruptured sinus of Valsalva aneurysm and stent implantation for aortic coarctation.

Ruptured sinus of Valsalva aneurysm is a rare anomaly and an associated coarctation of aorta is even rarer. A combination of such defects is traditionally treated surgically. The surgery is necessarily staged and done through different approaches. We report successful simultaneous transcatheter treatment of both these defects performed in the same setting in an acutely ill adult male patient with a good intermediate-term follow-up.

Myriad manifestations of Williams syndrome.

4 months male child presented with failure to thrive. On general examination child had normal O2 saturation with characteristic elfin facies. Further evaluation of the patient showed major manifestations of Williams syndrome in form of supravalvar aortic stenosis, branched pulmonary artery stenosis along with cardiomyopathy. Although the entity is known, this article shows comprehensive diagnostic workup with the aid of multimodality imaging techniques. The genetic diagnosis of Williams syndrome was confirmed using fluroscent in situ hybridisation techniques (FISH). In this patient most of the manifestations of elastin vasculopathy were noted in the form of involvement of ascending aorta, pulmonary arteries and myocardium. We also want to emphasis the importance of echocardiography in newborn patients with dysmorphic facies as Williams syndrome can be easily missed in neonatal period.

Percutaneous tricuspid valvotomy for pacemaker lead-induced tricuspid stenosis.

Permanent pacemaker lead-induced tricuspid regurgitation is extremely uncommon. We report a patient with severe tricuspid stenosis detected 10 years after permanent single chamber pacemaker implantation in surgically corrected congenital heart disease. The loop at the level of the tricuspid valve may have caused endothelial injury and eventually led to stenosis. Percutaneous balloon valvotomy for such stenosis has not been reported from India.

Transcatheter closure of hypertensive ductus with amplatzer post infarction muscular VSD occluder after percutaneous retrieval of embolized amplatzer duct occluder.

Transcatheter closure of a large hypertensive patent ductus arteriosus is challenging with scant data about it. Even more challenging is retrieval of an embolized Amplatzer duct occluder. We report successful closure of a 12 mm large ductus with the Amplatzer muscular VSD occluder (post myocardial infarction) after percutaneous retrieval of the embolized, largest available, 16/14 mm Amplatzer duct occluder.

Novel kissing catheter technique for foreign body retrieval.

We report a novel technique 'the kissing catheter technique' for retrieval of a broken catheter fragment in a patient undergoing closure of a patent ductus arteriosus.

Isolated single coronary artery (RII-B type) presenting as an inferior wall myocardial infarction: a rare clinical entity.

Isolated single coronary artery without other congenital cardiac anomalies is very rare among the different variations of anomalous coronary patterns. The prognosis in patients with single coronary varies according to the anatomic distribution and associated coronary atherosclerosis. If the left main coronary artery travels between the aorta and pulmonary arteries, it may be a cause of sudden cardiac death. We present multimodality images of a single coronary artery, in which the whole coronary system originated by a single trunk from the right sinus of Valsalva with inter-arterial course of left main coronary artery. This rare type of single coronary artery was classified as RII-B type according to Lipton's scheme of classification. A significant flow-limiting lesions were found in the right coronary artery that was successfully treated with percutaneous coronary intervention.

An extremely rare cause of Eisenmenger syndrome.

Persistent Truncus Arteriosus (PTA) is an extremely rare defect accounting for under 1% of all congenital heart disease, and over 85% of patients succumb to heart failure in infancy. We report a case of van Praagh type A3 PTA, which represents its rarest variant and accounts for only 8-10% of all patients with PTA. The presence of ductal stenosis in such cases is further exceedingly rare.

Three vanishing masses in the heart.

A 26 year primigravida, 8 days after delivering a still born baby presented with gradually progressive dyspnea from New York Heart Association (NYHA) class II to class III over a period of 1 week. 2D-Echocardiography showed severely compromised left ventricular systolic function with evidence of thrombi in three different chambers of the heart. All the clots disappeared after an effective anticoagulation therapy without any embolic complications. The hypercoagulable state of the peripartum period and the severe biventricular dysfunction led to the formation of clots. .