Factor VII Deficiency: A Rare Case Report.

Factor VII deficiency is a rare congenital coagulopathy. Prolonged prothrombin time with normal partial thromboplastin time indicates factor VII deficiency. For the definitive diagnosis, the specific factor VII level should be investigated.

Utility of intra-operative capnogram to detect branch pulmonary artery obstruction following total correction of tetralogy of Fallot.

Branch pulmonary artery obstruction is one of the prime reasons for re-operation in patients who have undergone repair for tetralogy of Fallot. Branch pulmonary artery obstruction may develop over a period of time due to dilation of right ventricular outflow tract or it may be caused by residual stenosis after inadequate repair. This may lead to differential lung perfusion causing morbidity.

Do we need a pulmonary artery catheter in cardiac anesthesia? - An Indian perspective.

There has been considerable controversy regarding the use of pulmonary artery catheter (PAC) in clinical practice. Some studies have indicated poor outcome in patients who were monitored with PAC. However, these studies, which have condemned the use of PAC, were conducted on patients in intensive care units, where the clinical scenarios with regard to patients' status are somewhat different as compared to those of a cardiac operating room.

PHACE/S syndrome: a syndromic infantile segmental hemangioma.

PHACES syndrome is a neurocutaneous disorder characterized by posterior fossa brain malformations, hemangiomas, cardiac anomalies and coarctation of aorta, eye anomalies ± sternal clefts. All reported cases are sporadic and notably common in females. The underlying cause is unknown.

Influence of colloid infusion on coagulation during off-pump coronary artery bypass grafting.

This study was conducted to determine the influence of colloid infusion on coagulation in patients undergoing off-pump coronary artery bypass grafting (OP-CABG). Thirty patients undergoing elective OP-CABG received medium molecular weight hydroxyethyl starch group I (MMW-HES 200/0.5), low molecular weight hydroxyethyl starch group II (LMW-HES 130/0.

Transesophageal echocardiaography evaluation of thoracic aorta.

Trans-esophageal echocardiaography is a sensitive, minimally invasive, diagnostic tool which gives real time functional image of the aorta. It helps in the diagnosis of pathologies of aorta like atherosclerosis, aneurysm and aortic dissection.

Unusual cardiac associations with Tetralogy of Fallot-a descriptive study.

We present a large single-center series (>2200 cases) operated for Tetralogy of Fallot (TOF). We analyzed the incidence of associated unusual and uncommonly described cardiac lesions and their diagnostic and therapeutic implications in TOF patients. This retrospective study was conducted by reviewing records of patients operated for TOF at a large tertiary care pediatric cardiac centre.

N-acetylcysteine does not prevent renal dysfunction after off-pump coronary artery bypass surgery.

Background And Objective: Coronary artery bypass graft surgery in high-risk patients may be associated with postoperative renal dysfunction. N-Acetylcysteine is a powerful antioxidant and has been used to prevent contrast-induced renal dysfunction. The efficacy of N-acetylcysteine in preventing postoperative renal dysfunction following off-pump coronary artery bypass graft surgery was studied.

Intraoperative transesophageal echocardiographic assessment of the mitral valve repair.

The use of intraoperative transesophageal echocardiography (TEE) in assessment of the mitral valve repair is well established. It has significantly contributed to the excellent results of mitral valvuloplasty in the current era. This article reviews various two-dimensional echocardiographic planes to assess the mitral valve apparatus, mechanisms of mitral regurgitation, different surgical techniques of repair, complications, and their recognition using TEE.

Postoperative management of pulmonary endarterectomy and outcome.

Pulmonary artery thromboendarterectomy (PTE) has been regarded as a promising, potentially curative surgical procedure. However, PTE is associated with specific postoperative complications, such as reperfusion pulmonary edema and right heart failure leading to a considerable mortality of 7-24%. Despite its limitations PTE is a better surgical alternative to lung transplantation which carries high morbidity and mortality.

A new association of multiple congenital anomalies/mental retardation syndrome with bradycardia-tachycardia syndrome: a case report.

Introduction: Congenital bradycardia-tachycardia syndrome is a rare disorder. Its association with multiple congenital anomalies/mental retardation (MCA/MR) syndrome is exceptional.

Case Presentation: We report a case of a new association of MCA/MR with bradycardia-tachycardia syndrome in an 18-year-old Indian man.

Vesicovaginal reflux: A case report.

Vesicovaginal reflux is a common cause of urinary incontinence in girls. A micturating cystourethrogram, which is the diagnostic investigation of choice, can demonstrate retrograde filling of the vagina during micturition and the complete emptying of the vagina at the end of micturition. Vesicovaginal reflux is a rare cause of gross hydrocolpos occurring without any anatomical obstruction.

Architecture of the left ventricle: insights for optimal surgical ventricular restoration.

The normal left ventricular shape has been defined as prolate ellipsoid. This shape is an adaptation to evolution. A knowledge of its unique macro and micro architecture forms the cornerstone in the understanding of its complex function.

Impact of preoperative mild renal dysfunction on short term outcome in isolated Coronary Artery Bypass (CABG) patients.

Background And Aim: It is well known that dialysis dependent renal failure increases the likelihood of poor outcome following cardiac surgery. But the results of CABG in patients with mild renal dysfunction are not clearly established. The aim of the study is to analyze the risk of preoperative mild renal dysfunction on outcome after isolated coronary surgery.

Supracardiac total anomalous pulmonary venous connection with a descending vertical vein.

The commonly used Darling classification for total anomalous pulmonary venous connection (TAPVC) consists of supracardiac, cardiac, infracardiac, and mixed types (Craig et al., Lab Invest 6:44-64, 1967). In supracardiac TAPVC, the common pulmonary vein drains superiorly into the left innominate vein, the superior vena cava, or the azygos vein by way of an ascending vertical vein.

Intra-operative trans-esophageal echocardiography in congenital heart disease.

Intra-operative trans-esophageal echocardiography (TEE) is an important monitoring and diagnostic tool used during surgery for repair of congenital heart disease. In several studies,TEE has been shown to provide additional intra-cardiac anatomic information. Its ability to be used intra- operatively before and after cardiac repair makes it a unique tool.

Severe mitral regurgitation due to left atrial aneurysm corrected by endoatrial patch plasty.

Congenital aneurysm of the body of the left atrium causing severe mitral incompetence is extremely rare. The cause of mitral incompetence has been attributed to a distortion of the mitral annulus by the aneurysm, or to coexisting pathology in the mitral valve apparatus. Surgical techniques to treat this condition include excision of the aneurysm along with mitral valve repair or replacement.

Aortic atresia with aortopulmonary window simulating common arterial trunk.

We describe the findings in a neonate with aortic atresia, interruption of the aortic arch between the carotid arteries, and aortopulmonary window. This unusual association changes the anticipated physiology, and helps in the survival. We emphasize the fact that this combination simulates common arterial trunk, both clinically and on echocardiography.

The efficacy of aprotinin in arterial switch operations in infants.

Background: In the present study we assessed whether aprotinin at a total dose (40,000 kallikrein inhibitor units (KIU)/kg) is effective in reducing postoperative blood loss and blood product requirement after arterial switch operations in infants.

Methods: A prospective, double-blind, randomized study, evaluated 50 infants who underwent arterial switch operations for transposition of great arteries. Patients were randomized into a placebo group, 25 patients who received normal saline and a treatment group, 25 patients who received 20,000 KIU/kg of aprotinin after induction of anesthesia, followed by 20,000 KIU/kg of aprotinin added to pump prime.

Gaucher's disease type III C: Unusual cause of intracardiac calcification.

We report a case of intracardiac calcification associated with oculomotor apraxia and corneal deposits in a 12-year-old girl, who presented with dyspnea on exertion, sinusitis, and epistaxis since the age of 6 years. Unusual presentation with multiorgan involvement prompted us to evaluate her in terms of metabolic/storage disorder. The bone marrow aspirate confirmed the diagnosis of Gaucher's disease.

Transfusion associated graft versus host disease in an immunocompetent individual following coronary artery bypass grafting.

Transfusion associated graft versus host disease (TA-GVHD) is a rare but commonly fatal complication of transfusion of cellular blood products, which usually occurs in immunosuppressed individuals following transfusion and subsequent engraftment of viable T lymphocytes. Very rarely it may arise in apparently immunocompetent individuals. The clinical syndrome consists of fever, skin rash, diarrhoea, hepatic dysfunction, and bone marrow aplasia.

Isolated atrioventricular discordance: surgical experience.

Background: Isolated ventricular inversion (atrioventricular discordance with ventriculoarterial concordance) is an extremely rare presentation of cyanotic congenital heart disease. The mode of presentation is akin to D-transposition of great arteries as systemic venous drainage and systemic arterial output connect to the same side of the cardiac septae, and pulmonary venous drainage and pulmonary arterial outflow to the opposite. Systemic oxygenation relies on intracardiac or extracardiac modes of mixing, as does survival, similar to transposition of the great arteries.

Severe pulmonary oedema following therapeutic embolization with Onyx for cerebral arteriovenous malformation.

Acute respiratory distress syndrome (ARDS) is characterized by sudden onset of respiratory distress, infiltrates on radiographs consistent with pulmonary oedema, hypoxaemia and increased work in breathing. Infiltrates on radiographs are bilateral, but may be patchy or diffuse and fluffy or dense. It is associated with absence of left heart failure and a PaO2/FiO2 ratio of < or =200.