Constantine Mavroudis

Coronary Artery Fistulas in Infants and Children: A Surgical Review and Discussion of Coil Embolization

BACKGROUND Coronary artery fistula (CAF) is a rare congenital anomaly that can be complicated by intracardiac shunts, endocarditis, myocardial infarction, or coronary aneurysms. Recent reports have emphasized the efficacy of percutaneous transcatheter techniques. The purpose of this article is to review a 28-year surgical experience with CAF as a standard for comparison and to discuss the emergence and efficacy of transcutaneous catheter coil embolization as an alternative form of therapy. METHODS From 1968 to 1996, 17 patients (age, 6 weeks to 16.5 years; mean age, 5.5 years) were diagnosed with CAF: 8 of 12 by echocardiography and 17 of 17 by cardiac catheterization. All patients with isolated CAF (n = 13) were asymptomatic despite significant clinical, electrocardiographic, and chest roentgenographic findings in 10. Sixteen had congenital CAF and 1 had acquired CAF after tetralogy of Fallot repair with injury of the anomalous left anterior descending coronary artery. Associated anomalies included tetralogy of Fallot (2), atrial septal defect (1), and patent ductus arteriosus (1). Nine fistulas originated from the right coronary artery and eight from the left. Drainage was to the right ventricle (9), right atrium (4), pulmonary artery (3), and left atrium (1). RESULTS All patients had a median sternotomy with epicardial or endocardial ligation. Cardiopulmonary bypass was used in 8; 1 of these (iatrogenic CAF) required distal internal mammary artery bypass graft. There were no operative or late deaths. Follow-up evaluation by physical examination (17), echocardiography (8), and catheterization (2) showed no evidence of recurrent or residual CAF. A retrospective review of the 16 available cine cardioangiograms showed that coil embolization was possible in, at most, 6 patients. CONCLUSIONS Early surgical management of CAF is a safe and effective treatment resulting in 100% survival and 100% closure rate. Transcatheter embolization is a reasonable alternative to standard surgical closure in only a very small, select group of patients. These surgical results should be considered the standard against which transcatheter techniques are compared.

Anomalous origin of the left coronary artery from the pulmonary artery: collective review of surgical therapy

Anomalous origin of the left coronary artery from the pulmonary artery is an extremely rare but potentially fatal congenital coronary anomaly. Prompt surgical reestablishment of a two-coronary system on diagnosis yields excellent results and allows progressive and nearly total myocardial recovery. Follow-up of all patients is required to assess the adequacy of repair and to exclude ongoing or recurrent myocardial insult.

Dexamethasone reduces the inflammatory response to cardiopulmonary bypass in children

BACKGROUND A randomized, prospective, double-blind study of 29 children was performed to evaluate the hypothesis that dexamethasone administration prior to cardiopulmonary bypass would decrease the inflammatory mediator release and improve the postoperative clinical course. METHODS Fifteen children received dexamethasone (1 mg/kg intravenously) and 14 (controls) received saline solution 1 hour prior to CPB. Serial blood analyses for interleukin-6, tumor necrosis factor-alpha, complement component C3a, and absolute neutrophil count were performed. Postoperative variables evaluated included temperature, supplemental fluids, alveolar-arterial oxygen gradient, and days of mechanical ventilation. RESULTS Dexamethasone caused an eightfold decrease in interleukin-6 levels and a greater than threefold decrease in tumor necrosis factor-alpha levels after CPB (p < 0.05). Complement component C3a and absolute neutrophil count were not affected by dexamethasone. The mean rectal temperature for the first 24 hours postoperatively was significantly lower in the group given dexamethasone than in the controls (37.2 degrees +/- 0.4 degrees C versus 37.7 degrees +/- 4 degrees C; p = 0.007). Dexamethasone-treated patients required less supplemental fluid during the first 48 hours (22 +/- 28 mL/kg versus 47 +/- 34 mL/kg; p = 0.04). Compared with controls, dexamethasone-treated children had significantly lower alveolar-arterial oxygen gradients during the first 24 hours (144 +/- 108 mm Hg versus 214 +/- 118 mm Hg; p = 0.02) and required less mechanical ventilation (median duration, 3 days versus 5 days; p = 0.02). CONCLUSIONS Dexamethasone administration prior to CPB in children leads to a reduction in the postbypass inflammatory response as assessed by cytokine levels and clinical course.

Revision of previous Fontan connections to total extracardiac cavopulmonary anastomosis: A multicenter experience.

BACKGROUND Conversion to total extracardiac cavopulmonary anastomosis is an option for managing patients with dysfunction of a prior Fontan connection. METHODS Thirty-one patients (19.9 +/- 8.8 years) underwent revision of a previous Fontan connection to total extracardiac cavopulmonary anastomosis at four institutions. Complications of the previous Fontan connection included atrial tachyarrhythmias (n = 20), progressive heart failure (n = 17), Fontan pathway obstruction (n = 10), effusions (n = 10), pulmonary venous obstruction by an enlarged right atrium (n = 6), protein-losing enteropathy (n = 3), right atrial thrombus (n = 2), subaortic stenosis (n = 1), atrioventricular valve regurgitation (n = 3), and Fontan baffle leak (n = 5). Conversion to an extracardiac cavopulmonary connection was performed with a nonvalved conduit from the inferior vena cava to the right pulmonary artery, with additional procedures as necessary. RESULTS There have been 3 deaths. Two patients died in the perioperative period of heart failure and massive effusions. The third patient died suddenly 8 months after the operation. All surviving patients were in New York Heart Association class I (n = 20) or II (n = 7), except for 1 patient who underwent heart transplantation. Early postoperative arrhythmias occurred in 10 patients: 4 required pacemakers, and medical therapy was sufficient in 6. In 15 patients, pre-revision arrhythmias were improved. Effusions resolved in all but 1 of the patients in whom they were present before revision. The condition of 2 patients with protein-losing enteropathy improved within 30 days. CONCLUSIONS Conversion of a failing Fontan connection to extracardiac cavopulmonary connection can be achieved with low morbidity and mortality. Optimally, revision should be undertaken early in symptomatic patients before irreversible ventricular failure ensues.

Congenital Heart Surgery Nomenclature and Database Project: anomalies of the coronary arteries

The extant nomenclature for coronary artery anomalies is reviewed for the purpose of establishing a unified reporting system. The subject was debated and reviewed by members of the STS-Congenital Heart Surgery Database Committee and representatives from the European Association for Cardiothoracic Surgery. All efforts were made to include all relevant nomenclature categories using synonyms where appropriate. The seven major categories of coronary artery anomalies are: anomalous pulmonary artery origins of the coronaries, anomalous aortic origins of the coronaries, congenital atresia of the left main coronary artery, coronary artery fistulas, coronary artery bridging, coronary aneurysms, and coronary stenosis. A comprehensive database set is presented which is based on a hierarchical scheme. Data are entered at various levels of complexity and detail, which can be determined by the clinician. These data can lay the foundation for comprehensive risk stratification analyses. A minimum database set is also presented, which will allow for data sharing and would lend itself to basic interpretation of trends. Outcome tables relating diagnoses, procedures, and various risk factors are presented.

Outcomes of 829 neonates with complete transposition of the great arteries 12–17 years after repair

OBJECTIVE Between 1985 and 1989, the surgical management of neonates with complete transposition (TGA) underwent a transition from atrial to arterial repair. We sought to examine the intermediate outcomes and their associated risk factors in neonates repaired during the era of transition. PATIENTS AND METHODS Twenty-four institutions entered 829 neonates age less than 15 days in a prospective study. Diagnosis was simple TGA (n=631), TGA with ventricular septal defect (VSD) (n=167), TGA with VSD and pulmonary stenosis (TGA/VSD/PS) (n=30), or TGA with PS (n=1). Repair was by arterial switch (n=516), atrial repair (Senning=175, Mustard=110) or Rastelli (n=28). Time-related events were analysed by parametric hazard function modeling and incremental risk factors for mortality, re-intervention, and late functional assessment were sought. RESULTS Survival estimates at 6 months, 5, 10, and 15 years are 85, 83, 83, and 81%, respectively. The hazard function for death after repair has two phases: an early rapidly declining phase and an ongoing constant one. Constant phase mortality is less likely after the arterial switch operation and in children with simple TGA. During follow up, at least one re-intervention was required in 167 children (pacemaker, n=35; percutaneous intervention, n=32; baffle re-intervention, n=27; re-operation, n=125). Freedom from re-intervention at 6 months, 5, 10 and 15 years is 93, 82, 77, and 76%, respectively. Of survivors, 87% have been followed up to the last 3 years, including an assessment of functional ability of 562 children (83%). Functional class 15 years after repair is class I in 76%, II in 22%, III in 2%. The proportion in functional class I decreased over time. Psychosocial deficits, especially learning disorders are prevalent. CONCLUSIONS Survival 15 years after TGA repair is good with most children functioning well, and results are best after an arterial switch operation. There is an ongoing risk of death that is less after the arterial switch operation. With the exception of Rastelli patients, the likelihood of survivors needing re-intervention after 5 years is low. There is need for improved neurodevelopmental outcomes.

Congenital Heart Surgery Nomenclature and Database Project: double outlet right ventricle

Double outlet right ventricle (DORV) is a type of ventriculoarterial connection in which both great vessels arise entirely or predominantly from the right ventricle. Although the presence of aortic-mitral discontinuity and bilateral coni are important descriptors, they should not serve as absolute prerequisites for the diagnosis of DORV. The morphology of DORV is encompassed by a careful description of the ventricular septal defect (VSD) with its relationship to the semilunar valves, the great artery relationships to each other, the coronary artery anatomy, the presence or absence of pulmonary outflow tract obstruction (POTO) and aortic outflow tract obstruction (AOTO), the tricuspid-pulmonary annular distance, and the presence or absence of associated cardiac lesions. The preferred surgical treatment involves the connection of the left ventricle to the systemic circulation by an intraventricular tunnel repair connecting the VSD to the systemic semilunar valve. This ideal surgical therapy is not always possible due to the presence of confounding anatomical barriers. A multitude of alternative surgical procedures has been devised to accommodate these more complex situations. A framework for the development of the DORV module for a pediatric cardiac surgical database is proposed.

Congenital Heart Surgery Nomenclature and Database Project: ventricular septal defect

The extant nomenclature for ventricular septal defect (VSD) is reviewed for the purpose of establishing a unified reporting system. The subject was debated and reviewed by members of the STS-Congenital Heart Surgery Database Committee and representatives from the European Association for Cardiothoracic Surgery. All efforts were made to include all relevant nomenclature categories using synonyms where appropriate. Four basic VSD types are described: Subarterial, Perimembranous, Inlet, and Muscular. A comprehensive database set is presented which is based on a hierarchical scheme. Data are entered at various levels of complexity and detail which can be determined by the clinician. These data can lay the foundation for comprehensive risk stratification analysis. A minimum database set is also presented which will allow for data sharing and would lend itself to basic interpretation of trends. Outcome tables relating diagnoses, procedures, and various risk factors are presented.

An empirically based tool for analyzing morbidity associated with operations for congenital heart disease

OBJECTIVE Congenital heart surgery outcomes analysis requires reliable methods of estimating the risk of adverse outcomes. Contemporary methods focus primarily on mortality or rely on expert opinion to estimate morbidity associated with different procedures. We created an objective, empirically based index that reflects statistically estimated risk of morbidity by procedure. METHODS Morbidity risk was estimated using data from 62,851 operations in the Society of Thoracic Surgeons Congenital Heart Surgery Database (2002-2008). Model-based estimates with 95% Bayesian credible intervals were calculated for each procedures average risk of major complications and average postoperative length of stay. These 2 measures were combined into a composite morbidity score. A total of 140 procedures were assigned scores ranging from 0.1 to 5.0 and sorted into 5 relatively homogeneous categories. RESULTS Model-estimated risk of major complications ranged from 1.0% for simple procedures to 38.2% for truncus arteriosus with interrupted aortic arch repair. Procedure-specific estimates of average postoperative length of stay ranged from 2.9 days for simple procedures to 42.6 days for a combined atrial switch and Rastelli operation. Spearman rank correlation between raw rates of major complication and average postoperative length of stay was 0.82 in procedures with n greater than 200. Rate of major complications ranged from 3.2% in category 1 to 30.0% in category 5. Aggregate average postoperative length of stay ranged from 6.3 days in category 1 to 34.0 days in category 5. CONCLUSIONS Complication rates and postoperative length of stay provide related but not redundant information about morbidity. The Morbidity Scores and Categories provide an objective assessment of risk associated with operations for congenital heart disease, which should facilitate comparison of outcomes across cohorts with differing case mixes.

Tracheal surgery in children: an 18-year review of four techniques.

OBJECTIVE Review the short- and long-term outcomes of a single institution experience in infants with congenital tracheal stenosis, comparing four different operative techniques used from 1982 through 2000. METHODS Hospital and clinic records of 50 infants and children who had surgical repair of congenital tracheal stenosis secondary to complete tracheal rings were reviewed. Age at surgery ranged from 7 days to 72 months (median, 5 months, mean 7.8+/-12 months). Techniques included pericardial patch tracheoplasty (n=28), tracheal autograft (n=12), tracheal resection (n=8), and slide tracheoplasty (n=2). All procedures were done through a median sternotomy with cardiopulmonary bypass. Seventeen patients had a pulmonary artery sling (35%), and 11 had an intracardiac anomaly (22%). RESULTS There were three early deaths (6% early mortality), two after pericardial tracheoplasty and one after autograft. There were six late deaths (12% late mortality), five after pericardial tracheoplasty and one after slide tracheoplasty. Length of stay (median) was 60 days (pericardial tracheoplasty), 28 days (autograft), 14 days (resection), and 18 days (slide). Reoperation and/or stent placement was required in seven patients (25%) after pericardial tracheoplasty, in two patients (17%) after autograft, in no patients after resection, and in one patient (50%) after slide tracheoplasty. CONCLUSIONS Our current procedures of choice for infants with congenital tracheal stenosis are resection with end-to-end anastomosis for short-segment stenoses (up to eight rings) and the autograft technique for long-segment stenoses. Associated pulmonary artery sling and intracardiac anomalies should be repaired simultaneously.