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.

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.

Situs Inversus Totalis: Single-Stage Anatomic Repair of Complex Congenital Heart Disease.

Transposition of the great arteries occurs rarely in patients with mirror image dextrocardia and situs inversus, while the combination with severe coarctation of the descending aorta (CoA) makes the anatomy even more unusual. Therefore, it is not surprising that a case with such unusual and complicated anatomy presents unique problems when a primary definitive correction is attempted. We report a patient with situs inversus totalis and complex congenital heart disease including transposition of the great arteries and severe CoA who underwent successful complete, single-stage, anatomic correction.

Rare multiple bronchial abnormalities in a patient with congenital heart disease.

Although many variations regarding lobar or segmental bronchial subdivisions have been described, abnormal bronchi originating from the trachea or main bronchi are relatively rare. These abnormalities can remain undetectable as they usually do not present with symptoms; however, they may pose major obstacles during surgery especially when accompanied by bronchial wall abnormalities.

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.

Thymic Cyst Popping Up in a Patient With Congenital Heart Disease.

The unusual case of a thymic cyst emerging and rapidly expanding, mimicking hence a right atrial aneurysm in an asymptomatic patient with congenital heart disease is presented.

Haddad Syndrome.

Congenital central hypoventilation syndrome (CCHS) causes predominantly sleep apnoea and is one of a growing number of inherited disorders characterised by autonomic nervous system dysfunction/dysregulation (ANSD). In association with Hirschsprung's disease (HSCR), it presents as Haddad's syndrome. We report a case of Haddad's syndrome complicated by sinus node dysfunction.

Operating the blues.

A 63-year-old man bearing most signs and symptoms (facial pigmentation, degenerative arthritis, and dark urine) pertinent to his known history of alkaptonuria underwent aortic valve replacement for critical aortic stenosis. Although rare, aortic stenosis is the most common cardiac manifestation of alkaptonuric ochronosis.

Pneumonectomy and contralateral metastasectomy through a single thoracotomy in a 9-year-old girl with a giant tumor.

A 9-year-old girl with a giant tumor of the right lung and an isolated metastasis of the left lower lobe underwent combined pneumonectomy and metastasectomy through means of a right thoracotomy. Her postoperative course was uneventful. The operative approach of a tumor of this scale and the concurrent contralateral metastasectomy are described and discussed.

Benign emptying of the post-pneumonectomy space: recognizing this rare complication retrospectively.

Patients presenting with a sudden drop in the pleural fluid level after a pneumonectomy in the absence of a recognizable bronchopleural fistula (BPF) have been classified as cases of benign emptying of the post-pneumonectomy space (BEPS). A retrospective study of 1378 pneumonectomies identified 4 cases of BEPS (0.29%). The patients were men; median age 64 years and all had undergone a right pneumonectomy. The median time at diagnosis was 31 days postoperatively and the median follow-up time was 31 months. None of the patients experienced a documented BPF or empyema. Although BEPS is an extremely rare complication, early recognition and close patient monitoring will prevent unnecessary interventional strategies.

Isolated congenital tricuspid insufficiency associated with right-sided congenital partial absence of the pericardium.

A unique case of successfully treated congenital tricuspid insufficiency (CTI) associated with partial absence of the right pericardium is described, and the clinical implications are discussed.

New xenograft valved conduit (Contegra) for right ventricular outflow tract reconstruction.

BACKGROUND: The well-known flaws of existing valved conduits for reconstruction of the right ventricular outflow tract (RVOT) continue to stimulate research for the elusive "perfect" conduit. In this study, we describe our experience with a glutaraldehyde-treated bovine jugular vein valved conduit (Contegra). METHODS: 55 years underwent implantation of a Contegra conduit. Diagnoses/procedures included repair of truncus arteriosus (2 patients), pulmonary atresia (3 patients), severe pulmonary insufficiency after prior repair of tetralogy of Fallot (9 patients), and replacement of degenerated valved conduit (1 patient). RESULTS: No operative deaths occurred. One patient required an early conduit replacement for unexplained valve thrombosis. The early postoperative mean transconduit pressure gradient was 7.7+/- 4.9 mm Hg. At a mean follow-up time of 18.5 +/- 6.9 months, all patients were asymptomatic with no discernible calcification in the valve or conduit or significant valve incompetence, while the mean transvalvular gradient remained low (11.1 +/- 4.5 mm Hg). CONCLUSION: The Contegra valved conduit is well suited for RVOT reconstruction, avoids the use of additional foreign material, and remains well functioning during early followup. Nonetheless, the long-term durability remains to be ascertained.

Right ventricular myxoma in a patient with tetralogy of Fallot.

INTRODUCTION: Cardiac myxoma is the most common primary cardiac tumour in adulthood and may present in the context of Carney's complex. PRESENTATION OF CASE: A 32-year-old male with a history of repaired tetralogy of Fallot in childhood was admitted with severe pulmonary valve regurgitation and a mobile mass in the right ventricle. The patient underwent pulmonary valve replacement and mass excision. Pathology examination showed myxoma. DISCUSSION: In the majority of cases myxomas originate in the atria, nevertheless they can also be found in a ventricular cavity. Myxoma is a prevalent feature of Carney's complex, an inherited, autosomal disease, characterised by multiple tumours in several organs. Tetralogy of Fallot has also been described in association with Carney's complex. CONCLUSION: Coexistence of tetralogy of Fallot with a cardiac ventricular myxoma in a patient not affected from Carney's complex or other familial syndrome.

Emerging surgical pathways of thoracotomy.

Thoracic incisions are the portals of choice for accessing thoracic organs. There are instances, however, that more than one incision are required at the same or a later stage, in order to access other, thoracic or extrathoracic, organs for more complicated procedures. Then again, a single thoracic incision may offer more than adequate access to extrathoracic organs and in selected cases becomes valuable surgical approach to organs of the upper abdomen or the contralateral hemithorax. The experience with this technique is discussed.

Postcardiotomy mechanical circulatory support in two infants with williams' syndrome.

Supravalvar aortic stenosis (SVAS) in patients with Williams' syndrome is often accompanied by coronary, pulmonary, and even myocardial lesions and therefore associated with increased perioperative morbidity and mortality. Extracorporeal membrane oxygenation (ECMO) provides reliable short-term mechanical circulatory support to patients, especially young, in acute postoperative cardiac failure when conventional means are ineffective. The incorporation of centrifugal pumps in these systems has made their use more efficient and less traumatic. We describe our experience of using the Levitronix CentriMag pump in two patients with Williams' syndrome who underwent surgical correction of supravalvular aortic stenosis.

Ross procedure in a child with Aspergillus endocarditis and bicuspid aortic valve.

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.