Acute Coronary Syndrome in a 9-Year-Old Girl With Homozygous Familial Hypercholesterolemia.

Familial hypercholesterolemia (FH) is a prevalent and underrecognized disorder. A young girl with previously undiagnosed homozygous FH presented with acute coronary syndrome. Severe coronary ostial stenosis and severe supravalvular aortic stenosis from atheromatous plaque was discovered.

Balancing anticoagulation and hemostasis in an infant with severe hemophilia A during cardiac transplantation: Review of the literature and development of a surgical protocol.

Hemophilia A is a disorder resulting in a deficiency of clotting factor VIII that can lead to life-threatening bleeding. Evidence-based guidelines for surgical interventions like cardiac surgery are limited. Anticoagulation is necessary for cardiac bypass, thus risk of bleeding in a patient with hemophilia is increased and requires careful attention to maintain hemostasis.

Isolated left subclavian artery with right aortic arch: case report and literature review of 50 cases.

Isolated left subclavian artery (ILSA) with right aortic arch is a rare vascular anomaly accounting for 0.8% in all right-sided aortic arch anomalies. We report a case of an isolated left subclavian artery with right aortic arch and a combination of subclavian as well as pulmonary steal in infant with Tetralogy of Fallot.

The long term results of the Ross procedure: The importance of candidate selection.

The Ross procedure has been considered in children as an optimal surgical procedure due to potential growth of the aortic annulus, lack of anticoagulation requirement, very low morbidity rate and excellent survival. Five-hundred-thirty-six (366 male, mean age 29.4 ± 11.

Incidence and outcome of acute kidney injury by the pRIFLE criteria for children receiving extracorporeal membrane oxygenation after heart surgery.

Background: Acute kidney injury (AKI) is common in patients treated with extracorporeal membrane oxygenation (ECMO). The RIFLE criteria demonstrate clinical relevance for diagnosing AKI and classifying its severity.

Objectives: To systematically define the incidence, clinical course and outcome of AKI using the pediatric pRIFLE criteria.

The short- and long-term effect of Blalock-Taussig shunt size on the outcome after first palliative surgery for cyanotic heart diseases.

Background And Objectives: The Blalock-Taussig (BT) shunt is regarded a safe and effective means increasing pulmonary blood flow for cyanotic heart conditions. The evaluation of shunt size for postoperative hemodynamics and until second-stage palliation remains difficult. Our objective is to compare the effect of different shunt sizes on short- and long-term outcomes after a BT shunt surgery.

Does single ventricle physiology affect survival of children requiring extracorporeal membrane oxygenation support following cardiac surgery?

Background: Improved survival with postoperative extracorporeal membrane oxygenation (ECMO) has expanded its application to children with single ventricle (SV) anomalies. We examine current-era outcomes of postoperative ECMO with special focus on patients with SV.

Methods: Demographic, anatomic, surgical, and support details of 100 consecutive children requiring postoperative ECMO (2007-2012) were included into multivariable regression models to identify factors affecting survival.

Results of rapid-response extracorporeal cardiopulmonary resuscitation in children with refractory cardiac arrest following cardiac surgery.

Objectives: Survival of children having cardiac arrest refractory to conventional cardiopulmonary resuscitation (CPR) is very poor. We sought to examine current era outcomes of extracorporeal CPR (ECPR) support for refractory arrest following surgical correction of congenital heart disease.

Methods: Demographic, anatomical, clinical, surgical and support details of children requiring postoperative ECPR (2007-12) were included in multivariable logistic regression models to determine the factors associated with survival.

Outcomes of Damus-Kaye-Stansel anastomosis at time of cavopulmonary connection in single ventricle patients at risk of developing systemic ventricular outflow tract obstruction.

Objectives: Children with various single ventricle anomalies are at risk of developing systemic ventricular outflow tract obstruction (SVOTO) following volume unloading with cavopulmonary connection (CPC). We aim to evaluate the value of Damus-Kaye-Stansel (DKS) anastomosis at the time of CPC in eliminating late SVOTO risk.

Methods: Retrospective review of single ventricle patients who underwent DKS concurrent with CPC between 1997 and 2012 was performed.

Left ventricular function and right ventricular pacing for isolated congenital heart block.

BACKGROUND: Right ventricular pacing has been the treatment of choice in patients with congenital complete atrioventricular block (CAVB). However, the effect of chronic right ventricular pacing on left ventricular function in young patients is still controversial. AIM: The aim of the study was to assess the change in left ventricular systolic function in young patients (age ≤20 years) paced for isolated CAVB and to identify possible predictors of left ventricular systolic dysfunction.

The fate of the neoaortic valve and root after the modified Ross-Konno procedure.

Objectives: In children with aortic valve disease associated with annular hypoplasia or complex multilevel left ventricular outflow tract obstruction, the Ross procedure, combined with a modified Konno-type aortoventriculoplasty, is advocated. We aim to examine the fate of the neoaortic apparatus and assess neoaortic valve function after the modified Ross-Konno procedure.

Methods: Forty-three patients, with a median age of 6 years, underwent the modified Ross-Konno procedure with a myectomy but without the use of a ventricular septal patch.

Current outcomes of the Glenn bidirectional cavopulmonary connection for single ventricle palliation.

Objectives: The Glenn bidirectional cavopulmonary connection (BCPC) is an established procedure in multistage palliation of various single ventricle (SV) anomalies. We aimed to report the current outcomes following BCPC and to examine risk factors affecting survival and progression to the next palliation stage.

Methods: Two hundred and twenty-seven consecutive children with variable SV pathologies underwent BCPC from 2002 to 2007.

Cardiac reoperations following the Ross procedure in children: spectrum of surgery and reoperation results.

Objectives: The Ross procedure is the preferred aortic valve replacement (AVR) choice in small children. Nonetheless, it is a complicated surgery and there are concerns that subsequent cardiac reoperations are exceptionally complex and associated with high morbidity and mortality. We examine the surgical spectrum and report outcomes of cardiac reoperations in patients who had undergone the Ross procedure during childhood.

A high-volume heart transplantation center in an Islamic country.

Cardiac transplants are performed sporadically or not at all in the majority of predominantly Muslim countries in the Middle East. We examined our experience in 76 patients who underwent heart transplantation between January 2005 and May 2010 in our center in Saudi Arabia. Excluded were 50 transplants performed between 1989 and 2004, due to incomplete data.

Older children at the time of the Norwood operation have ongoing mortality vulnerability that continues after cavopulmonary connection.

Objectives: Delayed first-stage palliation of children with hypoplastic left heart syndrome and related pathologies can be associated with poor outcomes because of development of progressive pulmonary vascular disease and volume load effects on the systemic ventricle and atrioventricular valve. We examine the current era's survival in this subgroup.

Methods: Fifty-five infants older than 2 weeks underwent the Norwood operation (2003-2007).

Outcomes and associated risk factors for mitral valve replacement in children.

Objective: We aim to report time-related outcomes following mitral valve replacement (MVR) in children and to identify factors affecting outcomes.

Methods: Clinical records from 307 children who underwent MVR between 1985 and 2004 were reviewed. Competing-risks methodology determined time-related prevalence of three mutually exclusive end-states: death, mitral reoperation and survival without subsequent MVR, and their associated risk factors.

Hybrid management strategy for percutaneous Fontan completion without surgery: early results.

Background: We report early results of surgical preparation and subsequent percutaneous Fontan completion strategy for the treatment of single-ventricle defects.

Methods: Two hundred twenty-seven patients underwent bidirectional cavopulmonary connection (BDCPC) between 2002 and 2007. Thirty-four patients had lateral tunnel created at time of BDCPC, fenestrated with 10 to 14 mm openings with the cardiac superior vena cava end patched to maintain BDCPC physiology.

Simultaneous aortic and mitral valve replacement in children: time-related outcomes and risk factors.

Background And Aim Of The Study: Valve replacement in children is problematic, and associated with high anticoagulation-related complications and increased reoperation requirements. Multiple valve replacement may further increase morbidity and worsen outcome. The results are reported of combined aortic valve replacement (AVR) and mitral valve replacement (MVR) in children.

The Ross procedure in children: preoperative haemodynamic manifestation has significant effect on late autograft re-operation.

Objectives: The Ross procedure is the aortic valve-replacement procedure of choice in children. Nonetheless, late autograft re-operation for dilatation and/or valve regurgitation is of concern. We examined whether preoperative haemodynamic manifestation (e.

Intermediate results following complex biventricular repair of left ventricular outflow tract obstruction in neonates and infants.

Objectives: Although the majority of infants with severe left ventricular outflow tract obstruction (LVOTO) can be managed with balloon or surgical aortic valvotomy, a more complex biventricular repair may be required in a subset of infants with multi-level obstruction, failure of or complication to prior intervention. In the presence of normal left ventricle size and inflow, the Ross procedure is applied in patients with/without ventricular septal defect (VSD), while the Yasui procedure is applied only in those with VSD. We report mid-term outcomes in a single institution.

The rastelli procedure for transposition of the great arteries: resection of the infundibular septum diminishes recurrent left ventricular outflow tract obstruction risk.

Background: The Rastelli procedure is the standard surgical treatment of d-transposition of great arteries (d-TGA), ventricular septal defect (VSD), and pulmonary stenosis. Late morbidity is significant due to recurrent left ventricular outflow obstruction (LVOTO), early conduit obstruction, and arrhythmias, with troublesome late mortality. To avoid recurrent LVOTO, we routinely enlarge the VSD and resect the infundibular septum before LV baffling to the aorta.

Mechanical valves versus the Ross procedure for aortic valve replacement in children: propensity-adjusted comparison of long-term outcomes.

Objective: We aimed to identify characteristics differentiating children undergoing aortic valve replacement by using mechanical prostheses versus the Ross procedure and to compare survival and the need for aortic valve reoperation after each procedure.

Methods: From 1983 to 2004, 346 children underwent aortic valve replacement (215 underwent the Ross procedure and 131 underwent placement of a mechanical prosthesis). Factors associated with procedure choice were used to construct a propensity score for use as a covariate in regression models to adjust for potential confounding by indication.