Andrew C. Chatzis

Etiology and management of chylothorax following pediatric heart surgery.

Abstract  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.

Minimal Invasive Coronary Artery Fistula Ligation

A coronary artery fistula was surgically ligated in a 38-year-old woman via a left anterior mini-thoracotomy without the use of cardiopulmonary bypass. In selected cases, this surgical approach can provide an excellent surgical exposure for coronary artery fistula ligation. It also offers an excellent cosmetic result and shorter hospital stay.

Successful Surgical Repair of a Giant Arteriovenous Fistula of the Coronary Arteries

Abstract  Large coronary arteriovenous fistulas (CAVF) cause severe symptoms and their repair involves the serious challenge of occluding the fistula while simultaneously preserving normal coronary flow. We present a case of a giant CAVF involving the right and left coronary arteries in a symptomatic adult patient and its successful surgical repair.

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.

Diversity of congenital cardiac defects and skeletal deformities associated with the Holt–Oram syndrome

INTRODUCTION The Holt–Oram syndrome is a rare congenital disorder involving the skeletal and cardiovascular systems. It is characterized by upper limb deformities and cardiac malformations, atrial septal defects in particular. PRESENTATION OF CASE Four consecutive patients 1–15 years old with the Holt–Oram syndrome presented over a 10 year span for surgical treatment of their cardiac maladies. The spectrum of the heart defects and skeletal deformities encountered in these patients are described and discussed. DISCUSSION The Holt–Oram syndrome is an autosomal dominant condition; however absence of the morphological features of the trait in close family members is not rare. Although patients are known to predominately present with atrial septal defects, other cardiovascular anomalies, including rhythm abnormalities, are not uncommon. Skeletal disorders vary as well. CONCLUSION Cardiovascular disorders, skeletal malformations and familial expression of the Holt–Oram syndrome, vary widely.

Early Left Ventricular Function Recovery After Trap‐Door Coronary Transfer Repair of ALCAPA in an Adult Patient

Abstract  Anomalous origin of the left coronary from the pulmonary artery (ALCAPA) is a rare congenital malformation, which may result in myocardial infarction, congestive heart failure, and sudden death if left untreated. Despite frequently advanced pathologic changes, there seems to be significant potential for the recovery of myocardial function in individuals with left ventricular dysfunction after the establishment of physiologic coronary circulation, particularly in the pediatric population. Reports of ALCAPA repair in adulthood are scarce and little information exists regarding the response of the left ventricle to revascularization in this age group. In this report, repair of ALCAPA in a significantly symptomatic adult patient with ventricular dysfunction is described, leading to an early recovery of left ventricular function.

Ross procedure in a child with Aspergillus endocarditis and bicuspid aortic valve : online article - case report

The case is presented of a previously healthy infant with a known asymptomatic bicuspid aortic valve who developed fungal endocarditis. The patient underwent aortic root replacement with a pulmonary autograft (Ross procedure). Cultured operative material revealed Aspergillus infection. The patient had an excellent recovery and remained well one year later.

Pulmonary valve replacement in patients with corrected tetralogy of Fallot

Introduction: Development of pulmonary insufficiency in patients with surgically corrected tetralogy of Fallot (TOF) may lead to severe right heart failure with serious consequences. We herein present our experience with pulmonary valve replacement (PVR) in these patients. Methods: From 2005-2013, 99 consecutive patients (71 males/28 females, mean age 38±8 years), underwent PVR after 7 to 40 (mean 29 ± 8) years from the initial correction. Seventy nine of the symptomatic patients presented in NYHA II, 14 in III and 2 in IV. All underwent PVR with a stented bioprosthetic valve, employing a beating heart technique with normothermic extracorporeal circulation support. Concomitant procedures included resection of aneurysmal outflow tract patches (n = 37), tricuspid valve annuloplasty (n = 36), augmentation of stenotic pulmonary arteries (n = 9), maze procedure (n = 2) and pulmonary artery stenting (n = 4). Results: There were 2 perioperative deaths (2%). One patient developed sternal dehiscence requiring rewiring. Median ICU and hospital stay was 1 and 7 days respectively. Postoperative echocardiography at 6 and 12 months showed excellent bioprosthetic valve performance, significant decrease in size of the right cardiac chambers and reduction of tricuspid regurgitation (TR) in the majority of the patients. At mean follow-up of 3.6 ± 2 years, all surviving patients remain in excellent clinical condition. Conclusion: Probability of reoperation for pulmonary insufficiency in patients with surgically corrected TOF increases with time and timely PVR by preventing the development of right heart failure is crucial for long-term survival. Current bioprosthetic valve technology in combination with the beating heart technique provides excellent immediate and short-term results. Further follow-up is necessary to evaluate long-term outcome.

Atrial mass: a myxoma?

A middle‐aged woman with a history of resected colorectal cancer and receiving chemotherapy presented with a right atrial mass and the provisional diagnosis of myxoma supported by echocardiography, computed tomography, and magnetic resonance imaging. Successful surgical removal revealed organized thrombus instead. Atrial thrombus may be mistaken for myxoma and long‐term intracardiac indwelling catheters can be thrombogenic.