Anomalous circumflex artery: a benign variant generating a malignant potential after valve surgery.

Coronary artery anomalies are a diverse group of congenital disorders presenting with highly variable clinical manifestations. The anomalous origin of left circumflex artery from the right coronary sinus following a retro-aortic trajectory is a well-recognized anatomic variation. Despite its benign course, it can prove lethal in association with valvular surgery. When single aortic valve replacement or combined with mitral valve replacement is performed, the aberrant coronary vessel may be compressed by or between the prosthetic rings triggering postoperative lateral myocardial ischemia. If left untreated, the patient is at risk of sudden death or myocardial infarction with its detrimental complications. Skeletonization and mobilization of the aberrant coronary artery is the most widely accepted intervention, but valve downsizing or concomitant surgical or transcatheter revascularization have also been described. However, large series are lacking from the literature. Therefore, no guidelines exist. This study is a thorough review of the literature concerning the aforementioned anomaly in association with valvular surgery.

Tying the knot: An uncommon complication.

Cardiothoracic procedures require continuous hemodynamic monitoring and a fair proportion of these require the insertion of a pulmonary artery catheter, known also as Swan-Ganz catheter. Given, however, the invasive nature of these procedures, unforeseen complications may ensue. Early recognition and appropriate handling are essential to minimize adverse outcomes.

Cavernous hemangioma of the right atrium.

A 42-year-old woman was diagnosed during the 3rd month of pregnancy with a large right atrial tumor. Three weeks after successful completion of a full-term pregnancy, she was admitted and underwent surgical removal of the tumor. Postoperative course was uneventful and pathology showed cavernous hemangioma. These neoplasms should be removed in order to prevent severe arrhythmias as also potential malignant transformation.

Etiology and management of chylothorax following pediatric heart surgery.

BACKGROUND: Chylothorax after congenital heart surgery (CHD) is a potentially challenging complication. The purpose of this study was to review our experience with the management of chylothorax following congenital heart surgery. METHODS: Between September 1997 and August 2006, of 1341 pediatric patients undergoing correction of congenital heart disease in our institution, 18 (1.3%) developed chylothorax postoperatively. Surgical procedures included tetralogy of Fallot repair in 10 patients, ventricular septal defect closure (one), atrial septal defect with pulmonary stenosis repair (one), Fontan procedure (three), coarctation of the aorta repair (one), aortopulmonary shunt (one), and ligation of patent ductus arteriosus in one patient. All patients followed a therapeutic protocol including complete drainage of chyle collection and controlled nutrition. Somatostatin was used adjunctively in six (33.3%) patients. Surgical intervention was reserved for persistent lymph leak despite maximal therapy. Following resolution of chylothorax, a medium-chain triglyceride diet was implemented for six weeks. RESULTS: There were no deaths. Fifteen patients (83.3%) responded to conservative therapy. Lymph leak ranged from 2.5 to 14.7 mL/kg per day for 8 to 42 days. Three patients with persistent drainage required thoracotomy with pleurodesis to achieve resolution, in two of which previously attempted chemical pleurodesis with doxycycline proved ineffective. Duration of lymph leak in this subgroup ranged from 15 to 47 days with 5.1 to 7.4 mL/kg per day output. CONCLUSIONS: Postoperative chylothorax is an infrequent complication of surgery for congenital heart disease and can occur even after median sternotomy in the absence of pathologically elevated venous pressure or Fontan circulation. Although hospitalization can be prolonged, conservative therapy is effective in most cases, while surgical pleurodesis proved successful in the refractory cases.

Hybrid procedures for complex congenital cardiac lesions.

BACKGROUND: We present an alternative treatment employing a hybrid approach used in 3 patients with congenital heart disease. The goal was to provide optimal therapy by minimizing the potentially harmful effects of methods that accompany conventional surgical procedures. METHODS: Two patients aged 4 and 6 months underwent beating-heart closure of a muscular ventricular septal defect (VSD) with an occluding device. In addition, an 8-year-old patient with supraaortic, main, and branch pulmonary artery (PA) stenosis underwent conventional surgical patch augmentation of the ascending aorta and the main PA and intraoperative stenting of the branch PA stenoses. RESULTS: No patient deaths occurred. One patient developed a postoperative pneumothorax. Median intensive care unit and hospital stays for the VSD patients were 1 and 5 days and for the other patients 2 and 20 days, respectively. At median follow-up of 25 months, all patients were well and had required no further interventions. CONCLUSIONS: Patients with muscular VSD can currently be treated with the hybrid approach. Intraoperative PA stenting in addition to conventional surgical repair can be performed safely and may be complementary in patients with complex lesions.

Mid-term results following surgical treatment of congenital cardiac malformations in adults.

The long term consequences of untreated of residual or recurrent lesions pose unique challenges in the growing population of adults with congenitally malformed hearts. In our unit, 335 patients aged from 18 to 72 years, with a mean age of 35 plus or minus 14 years, presented for correction of congenital cardiac disease from September, 1997, through December, 2006. Of the group, 42 (12.5%) had undergone one or more prior surgical procedures, 3 were admitted as emergencies, and a further 10 (3%) had suffered prior cardiac related complications. Symptoms had been noted by 181 patients (54%), and 42 (12.5%) had an established arrhythmia. Chromosomal anomalies were identified in 13 (3.8%), and diagnostic catheterisation was required in 201 (60%) patients. Of the overall group, 2 patients died early (0.6%). Complications occurred in 61 patients (18%), including atrial fibrillation, pneumothorax, postoperative haemorrhage, pericardial or pleural effusions requiring drainage, stroke, complete heart block, endocarditis, wound dehiscence, and peripheral neuropathy. The median length of stay in the intensive care unit and hospital were 2 and 7 days, respectively. Death occurred later in 2 further patients (0.6%), due to atrial fibrillation and pulmonary hypertension. At mean follow-up of 63 plus or minus 30 months, the majority of the remaining patients are well with resolution or significant improvement in their symptoms. Despite the long term deleterious effects of untreated, residual or recurrent congenital cardiac lesions in adults, therefore, we conclude that surgical correction can be achieved with low mortality and acceptable morbidity. Most significant complications are related to arrhythmias.

Surgical closure of an atrial septal defect in an 11-year-old girl with Silver-Russell syndrome.

Silver-Russell syndrome is a congenital disease characterized by severe growth retardation and variable dysmorphic features such as a small triangular face, micrognathia, asymmetry (usually of the limbs), syndactyly and clinodactyly. We report a rare case of a girl with extremely low body weight, short stature and an atrial septal defect, who underwent successful surgical repair of the heart abnormality.

Partial right-sided pericardial defect associated with congenital aortic valve disease.

Partial right-sided pericardial defect is an extremely rare congenital anomaly and is often associated with other congenital abnormalities. We describe a unique case of congenital aortic valve disease associated with right-sided pericardial defect. The clinical implications are discussed and a review of the literature is presented.

Emergency surgery for embolisation of Amplatzer device into the left atrium.

Occlusion of secundum atrial septal defects (ASD) by means of percutaneous transcatheter systems is increasingly used in clinical practice. A variety of different types and models of septal occluder devices are available nowadays, amongst which the Amplatzer device (Amplatzer Medical, Golden Valley, MN, U.S.A.), is regarded as one of increasing popularity. We report a case of attempted percutaneous transcatheter closure of a large ASD in an 11-year-old boy, complicated by device embolisation to the left atrium necessitating emergency surgery.

Right axillary artery cannulation for surgical management of the hostile ascending aorta.

Extensive aortic disease, such as atherosclerosis with aneurysms or dissections that involve the ascending aorta, can complicate the choice of a cannulation site for cardiopulmonary bypass. To date, the standard peripheral arterial cannulation site has been the common femoral artery; however, this approach carries the risk of atheroembolism due to retrograde aortic perfusion, or it is undesirable because of severe iliofemoral disease. Arterial perfusion through the axillary artery provides sufficient antegrade aortic flow, is more likely to perfuse the true lumen in the event of dissection, and is associated with fewer atheroembolic complications. From September 2000 through March 2004, 27 patients underwent right axillary artery cannulation for acute ascending aortic dissection (n = 16), ascending aortic aneurysm (n = 9), or coronary artery bypass grafting (n = 2). Direct artery cannulation was performed in the first 4 patients, and the last 23 patients were cannulated through a longitudinal arteriotomy via an 8-mm woven Dacron graft. Seventeen patients underwent hypothermic circulatory arrest and antegrade cerebral perfusion. Two patients died intraoperatively: one due to low cardiac output and one due to diffuse bleeding. One patient suffered mild right-arm paresthesia postoperatively, but recovered completely. Axillary artery cannulation was successful in all patients; it provided sufficient arterial flow, and there were no intraoperative problems with perfusion. In the presence of extensive aortic or iliofemoral disease, arterial perfusion through the axillary artery is a safe and effective means of providing sufficient arterial inflow during cardiopulmonary bypass. In this regard, it is an excellent alternative to standard femoral artery cannulation.

Combined repair of ascending aortic pseudoaneurysm and abdominal aortic aneurysm: in a patient with Marfan syndrome.

Pseudoaneurysms of the ascending aorta after the original inclusion/wrap technique of the Bentall procedure present a difficult surgical management problem and are associated with substantial morbidity and mortality. Patients with Marfan syndrome frequently develop aneurysms and dissections that involve multiple aortic segments. We present the case of a Marfan patient who successfully underwent repair of a giant ascending aortic pseudoaneurysm and concomitant repair of an abdominal aortic aneurysm. An aggressive surgical strategy followed by life-long cardiovascular monitoring is warranted in order to prolong the survival of these patients.