World Journal for Pediatric and Congenital Heart Surgery current issue

1. Left Atrial Myxoma in a Child: A Challenging Diagnosis of a Rare Lesion

   An eight-year-old child presented with congestive heart failure, blurred vision, and unexplained constitutional symptoms. An echocardiogram demonstrated a giant left atrial mass that obstructed the mitral valve inflow. After excision of the myxoma, the patient had an uneventful recovery.

2. Anomalous Coronary Arteries: Cardiovascular Computed Tomographic Angiography for Surgical Decisions and Planning

   Cardiovascular computed tomographic angiography (CCTA) provides an understanding of the three-dimensional (3D) coronary artery anatomy in relation to cardiovascular thoracic structures important to the surgical management of anomalous coronary arteries (ACAs). Although some ACA variants are not clinically significant, others can lead to ischemia/infarction, related acute ventricular dysfunction, ventricular arrhythmias, and sudden cardiac death. The CCTA is important to surgical decision making, as it provides noninvasive visualization of the coronary arteries with (1) assessment of origin, course, and termination of coronary artery anomalies in the context of 3D thoracic anatomy, (2) characterization of anatomy helpful for differentiation of benign versus hemodynamically significant variants, (3) identification of other cardiothoracic anomalies, and (4) detection of coronary artery disease. High-risk ACA anatomy in the appropriate clinical setting can require surgical intervention with decisions including minimally invasive versus open sternotomy approach, correction via reimplantation of a coronary artery, alteration of the ACA course without reimplantation, or bypass of an ACA. Given the rarity of ACA, there is limited data in the literature, and significant controversy related to the management issues. The management of ACA requires comprehensive clinical history, thorough assessment of cardiac function, and detailed anatomic imaging. Future studies will need to address the long-term outcome based on detailed assessment of original anatomy and surgical approach.

3. Tetralogy of Fallot With Absent Pulmonary Valve Syndrome, Right Aortic Arch, and Disconnected Left Pulmonary Artery

   We present a rare case of a two-week-old infant with tetralogy of Fallot , absent pulmonary valve syndrome , right aortic arch, and disconnected left pulmonary artery (LPA) whose origin was from ductal ligament adjacent to the left subclavian artery. One-stage surgical correction, including closure of ventricular septal defect (VSD), LPA reconstruction and reconnection to the pulmonary trunk, reduction in size of the right pulmonary artery (RPA), and right ventricular outflow tract reconstruction with valved conduit, was successfully performed with good clinical mid-term outcome.

4. Improved Long-Term Survival for Rheumatic Mitral Valve Repair Compared to Replacement in the Young
   Background:

   Mitral valve (MV) repair offers potential advantages over replacement in patients with rheumatic heart disease (RHD). We present the first long-term study that compares MV repair with replacement in children with RHD.

   Methods and Results:

   Single institute retrospective review of patients with RHD under 20 years of age, who underwent their first isolated MV surgery between 1990 and 2006. Of the 81 patients, 98% were Maori or Pacific Islander. The median age was 12.7 (3-19) years. The MV was repaired in 59%, a mechanical valve replacement (MVR) took place in 35% and bioprosthetic valve replacement in 6% of the patients. Follow-up data were available for 91.4% of the patients with mean follow-up of 7.6 years (range 0-19.4 years), a total of 620 patient years. Actuarial survival at 10 and 14 years for patients with MVR was 79% and 44%, compared to 90% and 90% for patients who underwent repair (P = .06). Actuarial freedom from late reoperation at 10 and 14 years for patients with MVR was 88% and 73%, compared to 76% and 76% for patients with repair (P = .52). Actuarial freedom from thrombotic, embolic, and hemorrhagic events at 10 and 14 years for patients with MVR was 63% and 45%, compared to 100% and 100% for patients with repair P < .01).

   Conclusion:

   This study shows that MV repair is superior to replacement for RHD in the young with follow-up to 19 years. Repair offers a survival advantage, greater freedom from valve-related morbidity, and long-term durability that equals that of MVR.

5. A Slight Modification of the Intra-atrial Conduit Fontan Procedure
6. Congenital Heart Disease in Mexico: Advances of the Regionalization Project

   Consistent with the mission of the World Society for Pediatric and Congenital Heart Surgery to promote health care for children with congenital heart disease all around the world, a Mexican Association of Specialists in Congenital Heart Disease (abbreviated in Spanish as AMECC) was created in Mexico in 2008. Our efforts were coordinated with those of the National Health Secretary with the objective being implementation of a national plan for regionalization of care for patients with congenital heart disease. To improve our knowledge related to technologic and human resources for management of congenital heart disease, we developed a national survey. Finally, a national database was created for collecting all Mexican centers’ information related to congenital heart disease care in order to quantify the advances related to the proposed plans. The database utilized international consensus nomenclature. The aim of this article is to show the sequence of our actions in relation to direct accomplishments and the current status of congenital heart disease care in Mexico. This article emphasizes the main aspects of these actions: regionalization project implementation, national survey results, and cardiovascular pediatric surgical database creation. Knowledge of outcomes related to successful actions would be useful for those countries that face similar challenges and may lead them to consider adoption of similar measures with the respective adjustments to their own reality.

7. Death by Late-Presenting Bochdalek Hernia in Infant Soon After Congenital Cardiac Repair

   Congenital diaphragmatic hernia (CDH) presenting beyond the neonatal period is commonly perceived to be rare. With reported frequencies of 2.6% to 20% of all CDH, it may be an overlooked cause of mortality. Variable symptomatology makes its diagnosis challenging. We report the sudden death of a 3-month-old patient shortly after hospital discharge following congenital heart surgery. Autopsy findings associated the patient’s demise with migrated abdominal contents in the chest through a Bochdalek hernia defect. No indications of CDH existed before hospital discharge. Relevant issues pertaining to congenital heart disease, CDH, and importance of autopsy in this context are discussed.

8. Congenital Heart Disease in Nigerian Children: A Multicenter Echocardiographic Study
   Background:

   Congenital heart disease (CHD) is among the leading causes of morbidity and mortality in childhood. We report on the spectrum of echocardiographically diagnosed CHD from three different centers across Nigeria.

   Methods:

   Over a period of 42 months, children who were referred for echocardiographic evaluation in the centers located in three large metropolitan cities were consecutively recruited if they were confirmed to have identifiable CHD. Data were collected on age, gender, and types of CHD and analyzed using SPSS 16 (Chicago, Illinois,).

   Results:

   A total of 605 children were recruited, their mean age was 2.1 ± 3.5 (range 0-17) years, and 296 (48.9%) were males. Nearly half (42.5%) had echocardiographic diagnosis of their CHD within the first year of life. Only 17% of the diagnoses were made in the neonatal age group. Acyanotic CHDs were more common than the cyanotic heart diseases (82.8% vs 17.2%). The most common CHD was ventricular septal defect (VSD; 46.6%) followed by patent ductus arteriosus (12.1%), atrial septal defect (8.7%), atrioventricular septal defect (8.2%), and tetralogy of Fallot (7.8%). More than half of the VSDs were perimembranous (55.1%) followed by outlet VSD (23.8%), muscular (10.7%), and inlet VSD (9.6%). Only 42 (6.9%) of the entire study population had definitive intervention.

   Conclusion:

   The VSD is the most prevalent CHD in Nigerian children. There is increasing awareness, availability, and use of diagnostic facilities as mirrored in the age distribution of the children. However, access to definitive surgery is poor and draws attention to the urgent need for affordable surgical facilities in the country.

9. Bilateral Branch Pulmonary Artery Banding as a Bridge to Decision/Preoperative Optimization of High-Risk Neonates With Hypoplastic Left Heart Syndrome

   We present our experience with bilateral branch pulmonary artery banding as a bridge to decision/optimization of hemodynamics, followed by standard Norwood stage I palliation in very high-risk infants with hypoplastic left heart syndrome.

10. Fetal Complete Common Atrioventricular Canal Defect: Spontaneous Closure of the Ventricular Septal Defect--In Utero Anatomic Evolution and Postnatal Outcomes
    Background:

    We describe in utero anatomic evolution and postnatal outcome of complete common atrioventricular canal defect (CCAVCD).

    Methods:

    Retrospective data on 31 fetuses with CCAVCD were analyzed. We reviewed prenatal and postnatal echocardiograms, karyotype, and postnatal outcomes.

    Results:

    A total of 20 fetuses had complete data, 18 with serial fetal echocardiograms and postnatal data and 2 terminations. At initial examination, isolated CCAVCD was seen in 12 (67%) fetuses while 6 (33%) were associated with heterotaxy syndrome. On follow-up, 4 fetuses (22%) had spontaneous closure of the inlet ventricular septal defect (VSD) component of the CCAVCD, seen both at 30 to 35 weeks of gestation and on postnatal echocardiograms. These 4 fetuses had previously demonstrated CCAVCD between 18 and 25 weeks of gestation. A total of 15 (83%) patients underwent operative correction, 10 with isolated complete atrioventricular septal defect and 5 with heterotaxy had surgical repair. Four infants in whom spontaneous intrauterine closure of the VSD component was observed had no VSD noted at surgery and underwent closure of primum atrial septal defect and repair of the left atrioventricular (AV) valve cleft.

    Conclusions:

    Our data demonstrate that CCAVCD diagnosed during fetal life is not a static anomaly. In our series, an inlet VSD less than 4 mm and Rastelli type A anatomy (AV valve attachment to septal crest) during second trimester may evolve during third trimester by formation of AV sulcus pouch and spontaneous closure of the VSD. To the best of our knowledge, this is the first study to report such anatomic evolution of CCAVCD in the fetus. This information is vital for appropriate counseling for expectant parents.

11. The Role of Databases in Improving the Quality of Care for Congenital Heart Disease
12. The Paediatric Cardiac Centre for Africa--Proceedings of the March 2012 Symposium

    The Pediatric Cardiac Centre for Africa (PCCA) was opened by national patron Mr Nelson Mandela on November 7, 2003. In 2008, the Centre’s international pediatric cardiac symposium was introduced as a learning forum for pediatric cardiac surgeons and cardiologists in the continent. The symposium has consistently grown in attendance and attracted distinguished leaders in the field. The 2012 symposium featured Dr. Thomas Spray of Children’s Hospital of Philadelphia, Dr. David Barron of Birmingham Children’s Hospital, and Dr. John Brown of Indiana University School of Medicine as guest speakers. Experience of the Fontan procedure, the small aortic root, hypoplastic left heart syndrome, right ventricular outflow tract reconstruction, transposition of the great arteries, and interrupted aortic arch were the highlights of the symposium. In the "African Corner," centers in South Africa, Ghana, and Angola presented work done from across the African continent.

13. Cardiac Myxoma With Prenatal Diagnosis

    The presentation of myxoma in the neonatal period is quite rare. We report the case of a female patient in whom two cardiac tumors were diagnosed prenatally. Surgery was performed at eight days of age, using cardiopulmonary bypass and circulatory arrest to facilitate excision of two polypoid tumors from within the right atrium. Pathology studies were consistent with myxoma. The postoperative course was satisfactory and the patient was discharged 14 days after surgery, at 22 days old.

14. Tetralogy of Fallot: Controversies in Early Management

    Management of the cyanotic neonate with tetralogy of Fallot (ToF) remains a challenging condition. Outcomes for single-stage repair of ToF have steadily improved over the past 30 years and the best results have been achieved with repair between 3 and 9 months of age. The traditional management of cyanotic neonates and small infants has been palliation with a Blalock–Taussig shunt, but this continues to carry a significant mortality that has remained relatively constant even in the contemporary series. This has led to the promotion of neonatal complete repair, but analysis of published outcomes would suggest that this also carries significant risk compared to repair at an older age. Low birth weight and small pulmonary arteries (PAs) remain the greatest independent risk factors. Right ventricular outflow tract (RVOT) stenting may offer an alternative approach to neonatal repair in high-risk neonates and allow for PA growth and delay of repair until the child reaches a safer age. A stratified approach to early management utilizing RVOT stenting in high-risk cases may lead to better overall outcomes.

15. Anatomically Corrected Malposition of the Great Arteries {S, D, L} With Left Juxtaposition of the Atrial Appendages in DORV: Influence on Surgical Approach

    The case of an infant with double outlet right ventricle with anatomically corrected malposition of the great arteries, bilateral infundibulum, and an echocardiographically routable ventricular septal defect (VSD) is presented. After numerous efforts to visualize the margins of the VSD, the best surgical exposure of the VSD was through the aorta. We believe this to be the first report of this phenomenon, which results from the left-sided position of the aorta and the presence of left juxtaposition of the atrial appendages.

16. European Congenital Heart Surgeons Association Presidential Address 2012
17. Congenital Division of the Left Atrium (Cor Triatriatum) in the Setting of Congenitally Corrected Transposition of the Great Arteries

    Congenital division of the left atrium (cor triatriatum) and congenitally corrected transposition of the great arteries are both rare congenital cardiac malformations; their coexistence is exceedingly rare with only two previous reports identified in the literature. This combination of lesions is characterized by a pressure-loaded morphologically left ventricle and a propensity for pulmonary edema dependent on the degree of pulmonary venous obstruction caused by the dividing left atrial shelf. The probable impact of this on the natural history and surgical decision making is discussed.

18. Severe Hyperkalemia During Cardiopulmonary Bypass: Etiology and Effective Therapy

    Hyperkalemia is considered a medical emergency as it can result in severe disturbances in cardiac rhythm and death. Although many causes of hyperkalemia exist, exogenous red blood cell transfusions are being recognized as the primary perioperative etiology. The authors report a case of severe intraoperative hyperkalemia associated with the use of allogeneic blood products (packed red blood cells), during a surgical mission to a developing country. The patient was undergoing repeat mitral valve replacement with cardiopulmonary bypass (CPB) and developed significant hyperkalemia with a serum potassium value of 9.9 mEq/L. Successful intraoperative therapies were instituted with a gradual reduction in the serum potassium value to 4.8 mEq, which allowed the patient to be weaned from CPB. The authors review the etiology of hyperkalemia in children including its relationship with allogeneic red blood cell transfusions and treatment modalities including specific therapies which can be instituted during CPB.

19. Isolated Left Common Carotid Artery Connected to the Pulmonary Artery: Where Was the Arterial Duct?

    A three-year-old boy was referred for persistent arterial duct. Transthoracic echocardiography showed a right aortic arch and an unusual Doppler flow in the arch vessels and the pulmonary artery. The tomodensitometry showed a right-sided aortic arch, with successive origin of the right common carotid, the right subclavian artery, and an aberrant (lusoria) left subclavian artery. The left common carotid took origin from the pulmonary trunk. During surgery, a fibrous cord independent from the anomaly was identified. An end-to-side anastomosis between the left carotid and the ascending aorta was done and the fibrous cord was divided. Was this fibrous cord a ductal ligament?

20. Isolated Anomalous Origin of the Right Coronary Artery From the Pulmonary Artery in an Asymptomatic 12-Year-Old Girl: Role of MRI in Depicting the Anatomy, Detecting the Ischemic Burden, and Quantifying the Amount of Left-to-Right Shunt

    Asymptomatic 12-year-old girl with a heart murmur underwent echocardiogram and suspicious continuous flow was detected in her interventricular septum. She underwent cardiac magnetic resonance (CMR) for further investigation. The CMR images demonstrated anomalous origin of the right coronary artery from the pulmonary artery. Adenosine stress perfusion scan showed an inducible perfusion defect in the right coronary artery and left circumflex artery territories. Flow quantification showed a left-to-right shunt with pulmonary to systemic blood flow ratio (Qp/Qs) of 1.25.

21. Reply to "A Slight Modification of the Intra-atrial Conduit Fontan Procedure" by Dr. F. Fantini