South African flag sign to a giant coronary artery aneurysm.

Background: Coronary arteritis leading to aneurysm is one of the unusual presentations of IgG4-related disease. Acute myocardial infarction as a complication of IgG4-related giant coronary artery aneurysm is even rarer. Case summary: We describe the case of a 56-year-old gentleman who presented to our institute with Canadian Cardiovascular Society (CCS) class III angina. His symptoms were persistent even with high-dose antianginal medications. He had an acute coronary syndrome two weeks back for which he was treated conservatively in a peripheral health centre. His 12-lead electrocardiogram at the time of the event was suggestive of high lateral ST-segment elevation myocardial infarction (South African flag sign). His transthoracic echocardiography showed mild left ventricular dysfunction and a large echogenic mass lateral to the left ventricle. Coronary angiography followed by cardiac computed tomography revealed a giant pseudoaneurysm of the proximal and mid-left anterior descending coronary artery. FDG-PET scan showed significant metabolic activity in the aneurysm wall and mediastinal lymph nodes suggesting active inflammation. IgG4-related coronary arteritis was suspected, and the patient underwent aneurysmectomy and coronary artery bypass (CABG) surgery. The histopathology of the resected segment showed diffuse IgG4-secreting plasma cells confirming the diagnosis. Discussion: Atherosclerosis is the most common cause of coronary aneurysms in adults. However, cardiologists should be aware of atypical causes like IgG4-related disease that can even present with acute coronary syndrome. Although multimodality imaging is beneficial during early evaluation, histopathological analysis is the cornerstone for the diagnosis of IgG4-related disease. The management involves both immunosuppressive medication and endovascular or surgical repair.

Concomitant Transthyretin Amyloidosis and Severe Aortic Stenosis in Elderly Indian Population: A Pilot Study.

BACKGROUND: Prevalence of both degenerative severe aortic stenosis (AS) and transthyretin cardiac amyloidosis (ATTR-CA) increases with age. Dual disease (AS+myocardial ATTR-CA) occurs in significant proportion of patients undergoing surgical aortic valve replacement (SAVR). OBJECTIVES: This study aimed to determine the prevalence of ATTR-CA in severe AS in the Indian population, identify noninvasive predictors of its diagnosis, and understand its impact on prognosis. METHODS: Symptomatic severe AS patients aged ≥65 years undergoing SAVR were enrolled. ATTR-CA diagnosis was based on preoperative 99m-technetium pyrophosphate (PYP) scan and intraoperatively obtained basal interventricular septum biopsy for myocardial ATTR-CA, and excised native aortic valve for isolated valvular ATTR-CA. Primary amyloidosis was excluded by serum/urine protein electrophoresis with serum immunofixation. RESULTS: SAVR was performed in 46 AS patients (age 70 ± 5 years, 70% men). PYP scan was performed for 32 patients, with significant PYP uptake in 3 (n = 3 of 32, 9.4%), suggestive of myocardial ATTR-CA. On histopathological examination, none of the interventricular septum biopsy specimens had amyloid deposits, whereas 33 (71.7%) native aortic valves showed amyloid deposits, of which 19 (57.6%) had transthyretin deposition suggestive of isolated valvular amyloidosis. Noninvasive markers of dual disease included low myocardial contraction fraction (median [interquartile range], 28.8% [23.8% to 39.1%] vs 15.3% [9.3% to 16.1%]; P = 0.006), deceleration time (215 [144 to 236] ms vs 88 [60 to 106] ms; P = 0.009) and global longitudinal strain (-18.7% [-21.1% to -16.9%] vs -14.2% [-17.0% to -9.7%]; P = 0.030). At 1-year follow-up, 2 patients died (4.3%); 1 each in myocardial ATTR-CA negative and positive groups (3.4% vs 33.3%; P = 0.477). CONCLUSIONS: Dual disease is not uncommon in India. Isolated valvular amyloidosis in severe AS is much more common.

Masson's Hemangioma Mimicking As Leaking Aortic Pseudoaneurysm: An Extremely Rare Presentation.

Intravascular papillary endothelial hyperplasia or Masson's tumor is a rare reactive disease of vascular origin characterized by exuberant proliferation of endothelial cells. Its importance lies in its ability to mimic a variety of diseases, both benign and malignant. Here, we present a unique case of Masson's tumor arising from the abdominal supraceliac aorta in a 32-year-old man initially misdiagnosed as leaking aortic pseudoaneurysm.

Use of autologous umbilical cord blood transfusion in neonates undergoing surgical correction of congenital cardiac defects: A pilot study.

BACKGROUND: Blood transfusion requirement during neonatal open heart surgeries is universal. Homologous blood transfusion (HBT) in pediatric cardiac surgery is used most commonly for priming of cardiopulmonary bypass (CPB) system and for postoperative transfusion. To avoid the risks associated with HBT in neonates undergoing cardiac surgery, use of autologous umbilical cord blood (AUCB) transfusion has been described. We present our experience with the use of AUCB for neonatal cardiac surgery. DESIGNS AND METHODS: Consecutive neonates scheduled to undergo cardiac surgery for various cardiac diseases who had a prenatal diagnosis made on the basis of a fetal echocardiography were included in this prospective observational study. After a vaginal delivery or a cesarean section, UCB was collected from the placenta in a 150-mL bag containing 5 mL of citrate-phosphate-dextrose-adenine-1 solution. The collected bag with 70-75 mL cord blood was stored at 2°C-6°C and tested for blood grouping and infections after proper labeling. The neonate's autologous cord blood was used for postcardiac surgery blood transfusion to replace postoperative blood loss. RESULTS: AUCB has been used so far at our institute in 10 neonates undergoing cardiac surgery. The donor exposure in age and type of cardiac surgery-matched controls showed that the neonates not receiving autologous cord blood had a donor exposure to 5 donors (2 packed red blood cells [PRBCs], including 1 for CPB prime and 1 for postoperative loss, 1 fresh frozen plasma, 1 cryoprecipitate, and 1 platelet concentrate) compared to 1 donor for the AUCB neonate (1 PRBC for the CPB prime). Postoperative blood loss was similar in both the groups of matched controls and study group. Values of hemoglobin, total leukocyte count, platelet counts, and blood gas parameters were also similar. CONCLUSIONS: Use of AUCB for replacement of postoperative blood loss after neonatal cardiac surgery is feasible and reduces donor exposure to the neonate. Its use, however, requires a prenatal diagnosis of a cardiac defect by fetal echo and adequate logistic and psychological support from involved clinicians and the blood bank.

Surgical Repair of Congenital Abdominal Aortic Aneurysm in a 1-year-old Child with Literature Review.

Reported here is a case of 1-year-old male child who presented with huge abdominal mass, which on radiological investigation was diagnosed as retroperitoneal pseudoaneurysm of the aorta. On exploration, it was found to be a true aneurysm of infrarenal abdominal aorta with inflow agenesis. Aneurysm was excised, and aorta was reconstructed with 10 mm Dacron graft. Postoperative computed tomography angiography showed patent graft with good distal runoff. Literature review revealed that only 26 cases of congenital abdominal aortic aneurysm had been reported so far. None of them had inflow agenesis which can give false impression of pseudoaneurysm on preoperative evaluation. The case highlights the utility of additional complimentary investigations such as Doppler study in clinching diagnosis and helping plan and execute successful treatment in the difficult diagnostic scenario.

Abernethy syndrome, a rare cause of hypoxemia: A case report.

Abernethy syndrome (congenital extrahepatic portosystemic shunt (CEPS II)) as an etiology of hepatopulmonary syndrome (HPS) is uncommon. The severe hypoxemia and its consequences become incapacitating for the patient. Early shunt closure resolves hypoxemia and clinical symptomatology and prevents irreversible changes in pulmonary vasculature.

Midterm outcome of primary arterial switch operation beyond six weeks of life in children with transposition of great arteries and intact ventricular septum.

BACKGROUND: We have previously reported our experience in primary arterial switch operation (ASO) in children more than six weeks with transposition of great arteries and intact ventricular septum (TGA/IVS). The upper age limit for performing an ASO in these children is not yet settled and reports regarding outcome of ASO in these children are few. In this prospective observational study, we report the midterm results of children with TGA-IVS older than six weeks undergoing primary ASO. METHODS: A total of 109 children aged more than 6 weeks with median age of 60 days (range 42-3,000 days), with regressed left ventricle underwent primary ASO. Extracorporeal membrane oxygenation was used in 20% (22 of 109) of them; 90.8% (99 of 109) of children who survived were prospectively followed, with a mean follow-up of 28 months (range 18-84 months). RESULTS: Two late deaths occurred, and survival in the remainder was estimated to be 98% at seven years. The incidence of aortic regurgitation (AR) was found to have a decreasing trend with freedom from AR approaching 100% by 34 months. The left ventricular shape and function returned to normal within one to three months following surgery. None of these children had any rhythm disturbances or evidence of myocardial ischemia. CONCLUSIONS: Primary ASO can be safely performed in children with regressed ventricle, irrespective of age with encouraging results. The midterm results of these children are comparable in terms of survival and freedom from complications associated with preserved ventricle.

Comparison of a waxy maize and a potato starch-based balanced hydroxyethyl starch for priming in patients undergoing coronary artery bypass grafting.

OBJECTIVE: Concerns have been raised about differences in the safety profile of potato- versus waxy maize-derived hydroxyethyl starch (HES). The objective of this study was to compare 2 HES solutions derived from 2 different source materials (potato versus waxy maize) for their dose-related effects on hemostasis and organ function when used to prime the cardiopulmonary bypass circuit (CPB). DESIGN: A prospective, randomized, controlled study. SETTING: Tertiary care center. PARTICIPANTS: Eighty patients undergoing coronary artery bypass grafting (CABG) on CPB. INTERVENTIONS: For priming the CPB circuit, the HESPRL group received 1000 mL of potato-derived balanced 6% HES 130/0.42 along with 500 mL of Ringer's lactate; the HESP group received 1,500 mL of potato-derived balanced 6% HES 130/0.42; the HESMRL group received 1000 mL of waxy maize-derived balanced 6% HES 130/0.4 along with 500 mL of Ringer's lactate, and the HESM group received 1500 mL of waxy maize-derived balanced 6% HES 130/0.4. MEASUREMENTS AND MAIN RESULTS: There were no significant differences in 24-hour mediastinal drainage, rate of re-exploration, blood product usage, coagulation parameters, and measures of pulmonary, renal, and hepatic function with respect to plant source of HES, when equivalent doses were used. Sonoclot activated clotting time (SonACT) was significantly higher and clot rate (CR) significantly lower at end of surgery (T1) and 24 hours after surgery (T2) in the HESP and HESM groups compared with the HESPRL and HESMRL groups. Compared with baseline, CR and platelet function were significantly lower at T1, PaO2/FIO2 ratio decreased significantly at T1 and T2, and serum bilirubin and transaminases increased significantly at T2 in all 4 groups. CONCLUSIONS: There was no significant difference in cumulative 24-hour mediastinal drainage when potato-derived balanced 6% HES 130/0.42 or waxy maize-derived balanced 6% HES 130/0.4 was used to prime the CPB circuit in patients undergoing CABG. In equal doses, both starches exerted the same effect on blood coagulation and pulmonary, renal, and hepatic function.

Aortoesophageal fistula in a child.

Aortoesophageal fistulae (AEF) are rare and are associated with very high mortality. Foreign body ingestions remain the commonest cause of AEF seen in children. However in a clinical setting of tuberculosis and massive upper GI bleed, an AEF secondary to tuberculosis should be kept in mind. An early strong clinical suspicion with good quality imaging and endoscopic evaluation and timely aggressive surgical intervention helps offer the best possible management for this life threatening disorder. Our case is a 10-year-old boy who presented to the pediatric emergency with massive bouts of haemetemesis and was investigated and managed by multidisciplinary team effort in the emergency setting.

Thrombus in right ventricular outflow tract: unique cause of refractory cyanotic spell.

Iron deficiency state in patients with cyanotic congenital heart disease can mimic as well as aggravate hyperviscosity symptoms. Correction of iron deficiency in these cases is expected to improve symptoms. We report an unexpected occurrence of refractory cyanotic spell in a child with tetralogy of Fallot due to thrombus in right ventricular outflow tract following intravenous iron sucrose therapy.

Surgical repair of D-TGA with an aortopulmonary window and ventricular septal defects / Correção cirúrgica da transposição das grandes artérias com janela aortopulmonar e defeitos do septo ventricular

D-Transposition of great arteries with an aortopulmonary window is a rare congenital anomaly. We describe a case of D-Transposition of great arteries with an aortopulmonary window and multiple ventricular septal defects in a 5-month boy who underwent successful surgical repair.

A transposição das grandes artérias com uma janela aortopulmonar é uma anomalia congênita rara. Descrevemos um caso de transposição das grandes artérias com janela aortopulmonar e múltiplos defeitos do septo ventricular em um menino de 5 meses submetido a tratamento cirúrgico com sucesso.

An electron microscopic study of left ventricular regression in children with transposition of great arteries.

Over the years the age limit for the arterial switch operation (ASO) is being redefined with increasing expertise and adoption of extra-corporeal membrane oxygenator (ECMO) in the surgical program. We conducted a study to see the differences in ultrastructural features in eight children with transposition of the great arteries, four with prepared and the remaining four with regressed left ventricle (LV) during the ASO. Children with prepared LV had prominent Z bands with uniform and round mitochondria, few fat vacuoles and minimal collagen in the background, whereas children with regressed LV had Z band disruption with non-uniform elliptical mitochondria and myofibrillary disarray and an abundance of fat vacuoles and collagen in the background. Children with regressed LV and abundance of collagen had a prolonged postoperative course. Collagen deposition in the LV may point to the situation where the postoperative course following ASO may be prolonged due to the increased time required for the regressed LV to increase its mass and to sustain the systemic circulation.

Primary arterial switch operation in children presenting late with d-transposition of great arteries and intact ventricular septum. When is it too late for a primary arterial switch operation?

OBJECTIVE: The surgical management of infants older than 2 weeks with d-transposition of great arteries and intact ventricular septum (IVS) is a matter of debate. Some studies have presented good results of primary arterial switch operation (ASO) in these children. The aim of this study was to assess the surgical outcome of the primary ASO in children with d-transposition of great arteries and IVS presenting beyond 6 weeks of age. METHODS: The clinical records of the children (more than 6 weeks age) with d-transposition of great arteries and IVS, who underwent primary ASO at our institute between January 2003 and June 2009 were reviewed. Left ventricular geometry and interventricular septal motion on the transthoracic cross-sectional echocardiogram were taken to assess the left ventricle preparedness. RESULTS: Fifty-five children (age ranging from 42 days to 9 years) with d-transposition of great arteries and IVS underwent primary ASO. The mean cardiopulmonary bypass time was 94.7±21.3 min, while mean aortic cross-clamp time was 53.2±8.1 min. Seven (13%) of these children died during their hospital stay. The children who had severely regressed left ventricle (banana-shaped left ventricular geometry) were operated with integrated extra corporeal membrane oxygenation-cardiopulmonary bypass (ECMO-CPB) circuit for left ventricular re-training. The children with regressed left ventricle required longer ventilatory time and inotropic support. Recovery of left ventricular geometry has taken 1-6 months depending on age at surgery. CONCLUSIONS: The children older than 6 weeks with d-transposition of great arteries and IVS can benefit from primary ASO with acceptable results. However, the need for mechanical support in some of the older patients may limit the widespread adoption of such a strategy.

Comparison of 48 h and 72 h of prophylactic antibiotic therapy in adult cardiac surgery: a randomized double blind controlled trial.

OBJECTIVES: To determine whether the duration of antibiotic prophylaxis influences the rate of surgical site infection in patients undergoing coronary bypass grafting or valve replacement. PATIENTS AND METHODS: Adult patients undergoing elective coronary artery bypass grafting (CABG) and valve surgery were included in this randomized double blind study. Between April 2007 and April 2008, 235 patients were randomly assigned to one of two groups using random number table and sealed envelope technique. The groups received prophylactic antibiotic therapy for either 48 h (the 48 h group) or 72 h (the 72 h group). These patients were monitored for surgical site infection. RESULTS: The mean age was 52.94 +/- 16.30 and 55.27 +/- 16.63 years, respectively, in the two groups. The incidence of co-morbid conditions as well as operative conditions was similar between the groups. During the study period 20 patients developed surgical site infections and 7 patients other infections. In modified treatment analysis, the infection rates were 7.6% (9 patients, n = 119) in the group receiving 48 h of prophylactic antibiotic therapy and 10.2% (11 patients, n = 108) in the group receiving 72 h of prophylactic antibiotic therapy, and the difference was statistically non-significant (P > 0.05). In the per protocol analysis the infection rates were 5% (5 patients, n = 100) in the group receiving 48 h of prophylactic antibiotic therapy and 8% (8 patients, n = 100) in the group receiving 72 h of prophylactic antibiotic therapy, and the difference was again statistically non-significant (P > 0.05). The results of Fisher's exact test revealed that the duration of surgery lasting for >5 h is an independent risk factor for surgical site infection. CONCLUSIONS: Forty-eight hours of a prophylactic antibiotic combination using a third-generation cephalosporin and an aminoglycoside is as effective as a 72 h regimen for preventing surgical site infection in patients undergoing CABG and valve surgery.

How to do it: repair of anomalous left coronary artery from pulmonary artery (ALCAPA) with coronary button transfer and Lecompte maneuver.

An alternative technique of coronary button transfer and Lecompte maneuver for anomalous left coronary artery (ALCAPA) arising from left lateral pulmonary sinus is described. This technique was used by us successfully in four patients aged 6 months to 3.5 years, weighing from 4.7 to 16 kg. The importance of trapdoor technique and Lecompte maneuver is discussed.

Surgical repair of D-TGA with an aortopulmonary window and ventricular septal defects.

D-Transposition of great arteries with an aortopulmonary window is a rare congenital anomaly. We describe a case of D-Transposition of great arteries with an aortopulmonary window and multiple ventricular septal defects in a 5-month boy who underwent successful surgical repair.

Surgical repair of multiple unruptured aneurysms of sinus of Valsalva.

Unruptured aneurysm of sinus of Valsalva (ASV) is a rare congenital anomaly. We describe a case of multiple unruptured ASV involving right and left aortic sinuses causing congestive cardiac failure in a 16-year-old boy who underwent successful surgical repair.

Application of stem cell technology for coronary artery disease at the All India Institute of Medical Sciences, New Delhi, India.

Stem cell technology is rapidly gaining popularity as a way to improve the prognosis of patients with coronary artery disease and heart failure. In this review, we systematically analyze the basis, methods, and results of stem cell technology for coronary artery disease at the All India Institute of Medical Sciences, New Delhi, India.