Chawki El-Zein - Academia.edu (original) (raw)

Papers by Chawki El-Zein

CHEST Journal, 2007

PURPOSE: Unroofing of anomalous coronary artery originating from opposite sinus of Valsalva has b... more PURPOSE: Unroofing of anomalous coronary artery originating from opposite sinus of Valsalva has become procedure of choice for this congenital lesion, with surgery performed in children as young as 2 years old. An increasing number of this anomaly is diagnosed in infancy with no clear indication for surgical repair. This paper reviews our experience with this anomaly, and focuses on its surgical management in infants. METHODS: Between April 2002 and February 2007, 8 patients underwent surgical repair of anomalous coronary artery arising from opposite sinus of valsalva and coursing between aorta and pulmonary artery. Patients' age varied from 2 months to 28 years with a mean of 11.7 ± 11.1 years. Surgical repair involved unroofing the intramural segment of anomalous coronary artery using cardiopulmonary bypass. RESULTS: Two patients were younger than one year old (group A), and 6 patients were older than one year (group B). The mean intensive care unit stay was 2.5 ± 0.7 days for group A and 2.8 ± 1.9 for group B. The mean hospital stay was 4 ± 1.4 days for group A and 4.3 ± 2.4 days for group B. There were no complications nor mortality. The mean follow up period is 14 ± 15.7 months with a range of 1-39 months. At the time of the last follow up, all patients were asymptomatic in NYHA class I and follow-up echocardiography on 6/8 patients showed wide open coronary ostium. CONCLUSION: Unroofing anomalous coronary artery arising from opposite sinus of valsalva can be done in infants with minimal morbidity and mortality. Longer follow up is needed to assess long-term results. CLINICAL IMPLICATIONS: Anomalous coronaries arising from the wrong sinus and coursing between aorta and pulmonary artery have been associated with sudden death. With improvement in echocardiographic imaging, more of these lesions are being diagnosed very early in infancy, raising the question of whether these patients should undergo surgery at an early age to prevent sudden death.

Congenitally Abnormal Aortic Valve causing Coronary Obstruction and Cardiac Arrest in Infancy

The Annals of thoracic surgery, 2020

Many instances of coronary artery anomalies are documented in the literature; however, the detect... more Many instances of coronary artery anomalies are documented in the literature; however, the detection and treatment of an asymmetric, large aortic cusp causing obstruction of a coronary ostium in a symptomatic infant remains unreported. We present a case of a two-month-old infant with an enlarged right coronary cusp obstructing the left coronary ostium, requiring emergent repair by relocating the left coronary button and reconstructing the sinus of Valsalva with autologous pericardium. This procedure preserved native aortic valve function, and the child remains asymptomatic months after discharge.

Pediatric aortic valve repair: Any development in the material for cusp extension valvuloplasty?

Journal of Cardiac Surgery

Post-Fontan pulmonary artery growth in patients with a bidirectional cavopulmonary shunt with additional antegrade pulsatile blood flow

Asian Cardiovascular and Thoracic Annals

Background Patients with antegrade pulmonary blood flow after a bidirectional cavopulmonary shunt... more Background Patients with antegrade pulmonary blood flow after a bidirectional cavopulmonary shunt (Glenn) may have better pulmonary artery growth. This study evaluated pulmonary artery growth in patients with and without prior additional pulsatile antegrade flow in a Glenn shunt at midterm follow-up after a Fontan procedure. Methods We reviewed 212 patients who had single-ventricle palliation in a 10-year period;103 (33 in pulsatile group 1 and 70 in nonpulsatile group 2) were selected for analysis. Data on demographics, procedures, perioperative course, and midterm follow-up after the Fontan procedure were compared. Echocardiography data were collected. Pulmonary artery sizes measured at cardiac catheterization and follow-up echocardiograms were used to calculate the Nakata index. Results Perioperative details were comparable in both groups, mean pulmonary artery pressure and systemic oxygen saturations were higher in group 1 compared to group 2. Venovenous collaterals were increas...

Technical Modifications that Might Improve Long-Term Outcome of the Ross Procedure in Children

The Annals of Thoracic Surgery

Outcomes From Three Decades of Infant and Pediatric Heart Transplantation

ASAIO Journal

Pulmonary hypertension and mitral regurgitation in an infant with an anatomically normal mitral valve

Cardiology in the Young

A full-term, female presented on her date of birth with severe pulmonary hypertension (PH) and mi... more A full-term, female presented on her date of birth with severe pulmonary hypertension (PH) and mitral regurgitation (MR), requiring veno-arterial extracorporeal membrane oxygenation. After the treatment, her PH and MR were resolved with no anatomic abnormality present. We propose a positive feedback loop of PH causing right ventricular dilation and interventricular septal shifts, worsening MR, and elevated left atrial, and potentially pulmonary, pressures.

A Unique Case of Middle Aorta Syndrome With a “Corkscrew” Descending Aorta

World Journal for Pediatric and Congenital Heart Surgery

Middle aortic syndrome is a rare anatomic defect of the descending aorta and presents with hypert... more Middle aortic syndrome is a rare anatomic defect of the descending aorta and presents with hypertension. We present a unique case with “corkscrew” configuration. This case highlights the need for evaluation with advanced imaging for diagnosis to facilitate surgical management and avoid hypertensive complications.

The Annals of thoracic surgery, 2017

Cor triatriatum sinister is a rare congenital lesion encountered in children. It consists of a fi... more Cor triatriatum sinister is a rare congenital lesion encountered in children. It consists of a fibromuscular membrane that separates the left atrium into two chambers resulting in a triatrial heart and often occurs with other structural cardiac anomalies. The acquired form is uncommon and has only been reported after orthotopic heart transplantation or as a complication of infective endocarditis in adults. These cases were mostly because of hypertrophied atrial tissue or suture lines and torsion of the atrium. We describe the first case of acquired cor triatriatum late after the Fontan procedure with successful surgical resection in a child.

ventricular outflow tract replacement in children Pericardial tissue valves and gore-tex conduits as an alternative for right

Background. There is still no perfect conduit for reconstruction of the right ventricular outflow... more Background. There is still no perfect conduit for reconstruction of the right ventricular outflow tract (RVOT) in children. Homografts are not always available in the appropriate size, and degenerate in a few years. This study evaluates the pericardial valve with Gore-Tex conduit as an alternative for RVOT construction. Methods. From January 1, 1993, to September 30, 1999, a pericardial tissue valve was inserted in all patients undergoing RVOT reconstruction or pulmonary valve replacement (PVR) who were large enough to accommodate a tissue valve. In patients without a native main pulmonary artery, a new technique was used to construct an RV-PA conduit out of a flat sheet of Gore-Tex, as Dacron frequently leads to stenosis. Data were collected by retrospective review, follow-up echocardiograms, and assessment by a single cardiologist. Results. There were 48 patients, 22 undergoing a PVR alone and 26 a RV-PA valved Gore-Tex conduit. Diagnosis included tetralogy of Fallot (n ‫؍‬ 25); truncus arteriosis (n ‫؍‬ 9); ventricular septal defect with PA (n ‫؍‬ 5); DORV (n ‫؍‬ 4); D-TGA with PS (n ‫؍‬ 2); and 1 each IAA with sub AS, VSD with PI, and PS s/p Ross procedure. Patient age ranged from 3 to 33 years and 98% were reoperations. The valve sizes ranged from 19 to 33 mm and the median hospital length of stay was 4 days. There were 2 (4.2%) perioperative and 1 (2.1%) late deaths, none related to the valve or Gore-Tex conduit. At a follow-up of 15 to 86 months (mean 43 ؎ 16 months), all remaining 45 patients are New York Heart Association class I, all valves are functional, and no patient has required valve or conduit replacement or revision; more importantly, echocardiogram revealed no significant valve or conduit stenosis (mean gradient 16 ؎ 8 mm Hg) and no evidence of regurgitation or structural degeneration. Conclusions. A pericardial tissue valve and Gore-Tex conduit provides a reliable alternative for RVOT reconstruction in pediatric patients. It is readily available, molds in the limited retrosternal space, and has outstanding intermediate results with no evidence of failure or deterioration up to 7 years after insertion.

Evolution of the surgical approach to congenitally corrected transposition of the great arteries

Seminars in thoracic and cardiovascular surgery. Pediatric cardiac surgery annual, 2015

The traditional surgical approach (physiologic repair) of congenitally corrected transposition of... more The traditional surgical approach (physiologic repair) of congenitally corrected transposition of the great arteries (ccTGA) attempts at restoring normal physiology by repairing the associated lesions. It fails to address the most serious anatomic abnormality, mainly ventriculoarterial discordance, and results in less than optimal long-term outcomes. Anatomic repair was introduced to incorporate the left ventricle into the systemic circulation. The excellent short-term and intermediate results of the double switch operation and its modifications make it the procedure of choice for the treatment of ccTGA.

hypoplastic left heart syndrome Repair of symptomatic neoaortic aneurysm after third-stage palliation for

Staged Biventricular Repair for Neonates With Left Ventricular Outflow Tract Obstruction, Ventricular Septal Defect, and Aortic Arch Obstruction

The Annals of Thoracic Surgery, 2014

The purpose of this study is to evaluate clinical outcomes of neonates who underwent a Norwood op... more The purpose of this study is to evaluate clinical outcomes of neonates who underwent a Norwood operation as a first step of a planned biventricular repair and the impact of associated risk factors. A retrospective cohort study was performed on all neonates (n = 44) undergoing the Norwood operation as the first stage of a biventricular (Norwood-Rastelli) repair from January 2000 to December 2012 at a single center. Multivariable analysis was performed to identify predictors of survival. Stage one mortality was 9%. The interstage survival for nonsyndromic and syndromic patients was 100% versus 46%, respectively (p &amp;amp;amp;amp;amp;amp;amp;amp;amp;lt; 0.001). Twenty-four patients (55%) underwent biventricular completion repair with no mortality. Freedom from reintervention after biventricular completion was 53% at 6 years. The overall survival for nonsyndromic patients versus syndromic patients was 86% versus 43%, respectively (p = 0.01). Genetic syndromes and prematurity were significant predictors of interstage mortality on multivariable analysis. Staged biventricular repair for patients with complex left ventricular outflow tract obstruction, ventricular septal defect, and aortic arch obstruction can be achieved with excellent outcomes for neonates without genetic syndromes. The staged approach is associated with longer time to reintervention after the biventricular completion.

World Journal of Surgery, 2008

Several major advances in the field of neonatal cardiac surgery have improved the outcome of thes... more Several major advances in the field of neonatal cardiac surgery have improved the outcome of these severely ill patients. Among the preoperative advances are improvement in the management of patients with single ventricle equivalents, prenatal diagnosis, and interventional procedures. Intraoperative advances include better understanding of the inflammatory process caused by cardiopulmonary bypass and ways to manage it, newer techniques for treating hypoplastic left heart syndrome and aortic valve disease, and primary repair of complex lesions. Postoperative refinements in managing pulmonary hypertension, low cardiac output state, and ventricular assist devices have decreased surgical mortality and morbidity. Although his primary interest has been adult cardiac surgery, Dr. Geha has contributed directly and indirectly to these advances. C. el-Zein Á M. N. Ilbawi

Tetralogy of Fallot With Atrioventricular Septal Defect: Surgical Strategies for Repair and Midterm Outcome of Pulmonary Valve-Sparing Approach

Pediatric Cardiology, 2013

Repair for tetralogy of Fallot (TOF) with complete atrioventricular septal defect (CAVSD) has bee... more Repair for tetralogy of Fallot (TOF) with complete atrioventricular septal defect (CAVSD) has been reported with good early and intermediate outcomes. Morbidity, however, remains significantly high. To date, repair of CAVSD/TOF using a pulmonary valve-sparing technique (PVS) and freedom from valve reoperation are not well defined. A study was undertaken to investigate outcomes. This study was conducted in as a retrospective investigation. Between January 1988 and December 2008, 13 consecutive patients with CAVSD/TOF were identified, and their records were reviewed retrospectively. Of these 13 patients, 9 had Rastelli type C CAVSD. Trisomy 21 was present in 9 cases (69 %; 7 with type C). Five patients had received a systemic-to-pulmonary shunt (SPS) before complete repair at a mean age 1.7 ± 0.6 months. All the patients survived until complete repair. At complete CAVSD/TOF repair, AVSD was corrected with a two-patch technique in all patients. For eight patients (61.5 %), PVS was used. The remaining five patients had transannular patch (TAP) repair. The mean age at complete repair was 6.3 ± 2.4 months. At complete repair, the mean cardiopulmonary bypass time was 173.5 ± 30.6 min, and the cross-clamp time was 134.7 ± 28.8 min. There was one hospitalization and no late deaths. The median follow-up period was 9.2 years [interquartile range (IQR), 4.7-13.3 years]. The actuarial survival was 90.0 ± 9.5 % at 1 year, 90 ± 9.5 % at 5 years, and 90 ± 9.5 % at 8 years. Of the 12 survivors, 6 had some reintervention during the follow-up period. Within the first 11 years after complete repair, two patients underwent left atrioventricular (AV) valve repair, and one patient had right AV valve repair. Two patients had residual VSD closure. Four patients underwent the first right ventricular outflow tract (RVOT) reintervention for critical insufficiency or stenosis at a mean interval of 6 ± 21) months. One patient had a second RVOT reoperation. Findings showed that CAVSD/TOF with PVS was related to significantly higher freedom from RVOT reintervention (100 % at 1, 5, and 8 years compared with 80 ± 17.9 % at 1 year, 60 ± 21.9 % at 5 years, and 40 ± 21.9 % at 8 years for CAVSD/TOF using TAP; P &amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;lt; 0.05). No patient who underwent PVS had left ventricular outflow tract obstruction requiring reoperation. Overall freedom from any reintervention was 90.9 ± 8.6 % at 1 year, 71.6 ± 14.0 % at 5 years, and 53.7 ± 8.7 % at 8 years in this group of patients. Correction of TOF with CAVSD can be performed at low risk with favorable intermediate-term survival and satisfactory freedom from reoperation. Use of TAP can be avoided in almost two thirds of patients and may influence freedom from early RVOT reintervention.

Pediatric Cardiology, 2008

Anomalous origin of the left coronary artery is a well-known cause of sudden death. Noncompaction... more Anomalous origin of the left coronary artery is a well-known cause of sudden death. Noncompaction of the ventricular myocardium is a cardiomyopathy characterized by prominent trabeculae and deep intertrabecular recesses. Both anomalies are rare. We report the case of a child with both anomalous origin of the left coronary artery from the right sinus of Valsalva and noncompaction of the left ventricular myocardium found during an evaluation for Kawasaki's disease.

Pediatric Cardiology, 2011

Aortic atresia-mitral stenosis (AA-MS) has been implicated as a determinant of outcome after Stag... more Aortic atresia-mitral stenosis (AA-MS) has been implicated as a determinant of outcome after Stage-1 palliation (S1P) in hypoplastic left heart syndrome (HLHS). Studies evaluating the association of AA-MS with ventriculo-coronary connections (VCC) and mortality report conflicting results. The significance of VCC, myocardial protection, and shunt strategy after S1P has yet to be determined. Between January 2005 and July 2009, 100 neonates with HLHS underwent S1P. Mitral and aortic valves and presence of VCC were assessed. Antegrade continuous cold blood cardioplegia was administered throughout the vast extent of the neo-aortic reconstruction. A right ventricle-topulmonary shunt was used for an ascending aortic diameter of 0.6 mm/kg or less. Survival analysis was performed to determine predictors and assess impact of AA-MS and VCC on hospital and interstage mortality. Twenty-seven (of 100) patients had AA-MS. The mean age and weight at S1P were 6.5 ± 2.8 days and 3.09 ± 0.47 kg, respectively. VCC were found in 56% of AA-MS. Twenty-two had Norwood-Sano, 3 had classic Norwood, and 2 had hybrid S1P. VCC were associated with AA-MS, endocardial fibroelastosis, and ascending aortic size\2 mm (P \ 0.05) but not higher mortality (P = ns). Operative and interstage survival for AA-MS after S1P was 85.2 and 71%, respectively (not statistically different compared to all other subtypes; P = ns). Actuarial survival after S1P at 1, 3, 6, 12, and 36 months was 92.9 ± 4.9, 78.6 ± 7.8, 75 ± 8.2, 71.3 ± 8.3, and 71.3 ± 8.3%, respectively. Intact atrial septum and post-S1P renal dysfunction (P \ 0.05) were independent predictors of hospital and interstage mortality. In patients with HLHS, AA-MS carries no survival disadvantage after S1P during the hospital and interstage period regardless of VCC. Intact atrial septum and post-S1P renal dysfunction predict early and interstage mortality. Myocardial protection and shunt strategy might influence the outcome in this HLHS variant.

Pediatric Cardiology, 2010

Pericardial effusion in neonates is a rare occurrence associated with malpositioning of central v... more Pericardial effusion in neonates is a rare occurrence associated with malpositioning of central venous catheters. This report describes a case of pericardial effusion in which echocardiographic determination of line position, typically considered one of the most reliable means of placement verification, was misleading. The infant ultimately did well after pericardiocentesis, with complete resolution of symptoms and no further complications.

Fate of Ventricular and Valve Performance Following Early Bidirectional Glenn Procedure After Norwood Operation Controlled for Hypoplastic Left Heart Syndome Anatomic Subtype

Pediatric Cardiology, 2014

The Norwood operation (NO) with a right ventricle (RV)-to-pulmonary artery (PA) shunt (NRVPA) is ... more The Norwood operation (NO) with a right ventricle (RV)-to-pulmonary artery (PA) shunt (NRVPA) is reportedly associated with early hemodynamic advantage. Shunt strategy has been implicated in ventricular function. Outcomes after NRVPA compared with classic procedure as part of a strategy involving early bidirectional Glenn (BDG) procedure were analyzed with reference to RV, tricuspid, and neoaortic valve performance. Between January 2005 and December 2010, 128 neonates with hypoplastic left heart syndrome (HLHS) underwent NO. Controlled for aortic/mitral stenosis (AS-MS) subtype, 28 patients underwent NRVPA (group A), and 26 patients had classic procedure (group B). The patients with a non-HLHS single-ventricle anatomy and those who had undergone a hybrid approach for HLHS were excluded from the study. The mean age at NO was 6.8 ± 3.5 days in group A and 6.9 ± 3.6 days in group B. Transthoracic echocardiographic evaluation (TTE) after NO (TTE-1) at the midinterval between NO and BDG (TTE-2), before BDG (TTE-3), before Fontan (TTE-4), and at the last follow-up evaluation (TTE-5) was undertaken. Cardiac catheterization was used to assess hemodynamic parameters before the Glenn and Fontan procedures. The operative, interstage, and pre-Fontan survival rates for AS-MS after NO were respectively 88.1 % (90.3 % in group A vs. 84.7 % in group B; p = 0.08), 82.5 % (82.7 % in group A vs. 81.8 % in group B; p = 0.9), and 80.7 % (79.5 % in group A vs. 81.8 % in group B; p = 0.9). The median follow-up period was 39.6 months (interquartile range 2.7-4.9 months). The RV global function, mid- and longitudinal indexed dimensions, fractionated area change before BDG (TTE-1, TTE-2, TTE-3) and after BDG (TTE-4, TTE-5), and right ventricular end-diastolic pressure did not differ statistically between the groups (p &amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;gt; 0.05). No statistically significant difference in tricuspid or neoaortic intervention was found between the groups (p &amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;gt; 0.05). Controlled for the AS-MS HLHS subtype, shunt strategy showed no midterm survival or hemodynamic (ventricular or valve) impact. At midterm, the follow-up need for neoaortic or tricuspid valve surgical intervention was not affected by shunt selection. The structural ventricular adaptation after reversal of shunt physiology was irrespective of shunt strategy.