Complex Cyanotic Congenital Heart Disease Research Articles

Anesthesia Management in Blalock-Taussig Shunt Procedure

Background: The systemic to pulmonary artery shunts are done as palliative procedures for complex cyanotic congenital heart diseases. Blalock-Taussig shunt (BT shunt) provide regulated blood flow to the lungs allowing growth of pulmonary arteries until the patient reaches proper age and body weight suitable for definitive corrective repair. BT shunts are first line management in patients with critical cyanotic conditions.Case: A 12-month-old boy diagnosed with PA-VSD subaortic, L-R shunt PDA and critical PDA stenosis experienced a recurrent spell condition with the lowest oxygen saturation 40%, underwent urgent BT shunt surgery. Oxygen saturation increases to 80-85% after shunt procedure.Discussion: Anesthesia management includes optimizing preoperative condition and patient hydration state, providing balance anesthesia during surgery, maintaining balance of pulmonary and systemic blood flow. High oxygen fraction can be given to maintain oxygen saturation before BT shunt anastomosis. Mechanical ventilation, heart rate with sinus rhythm, preload and contractility is maintained to obtain normal cardiac output. After BT shunt anastomosis, the oxygen fraction is reduced with a saturation target of 70–85%. Postoperative management includes anticoagulant administration and monitoring postoperative complications. The patient developed complications of increased pulmonary blood flow postoperatively and was admitted to the PICU for 3 days. The patient was discharged in good condition from ward on day 7.Conclusion: Understanding the physiology of heart defects and perioperative management determine the success of BT shunt surgery, reducing patient morbidity and mortality. Optimizing intraoperative and postoperative oxygen delivery with oxygenation targets PaO2 40-45 mmHg and saturation 70-80% reflects the balance of pulmonary blood flow and systemic blood flow (Qp:Qs=0.7-1.5:1).

Aortic valve replacement in a 41-year-old woman with uncorrected tetralogy of Fallot, pulmonary atresia, and major aortopulmonary collateral arteries: a case report

BackgroundTetralogy of Fallot (TOF) is a complex cyanotic congenital heart disease. As most patients with TOF undergo palliative or radical surgical repair during childhood, cardiac surgery under cardiopulmonary bypass (CPB) for adult survivors with unrepaired TOF is exceedingly rare.Case presentationA 41-year-old woman with unrepaired TOF, pulmonary atresia (PA), and major aortopulmonary collateral arteries (MAPCAs) developed acute infectious endocarditis (IE). As vegetation gradually increased despite intravenous antibiotic administration, she was scheduled for urgent aortic valve replacement under CPB. Pulmonary blood flow was primarily provided by the MAPCAs originating from the descending aorta. Intra-aortic balloon occlusion for MAPCAs was performed to ensure a bloodless surgical field. Aortic valve replacement was successful.ConclusionAn adult with uncorrected TOF developed acute IE and subsequently had successful cardiac surgery under CPB. Understanding TOF physiology with PA and MAPCAs, particularly pulmonary blood flow through MAPCAs, is crucial.

SAT631 EPAS1 Mutations In Three Adjacent Nucleotides Resulting In Different Phenotypes

Abstract Disclosure: M. Alswailem: None. B. Alghamdi: None. L.A. Alobaid: None. H. Al-Hindi: None. M. Aldawish: None. O. Alsagheir: None. A.S. Alzahrani: None. EPAS1 (HIF2a) mutations have been described in patients (pts) with paragangliomas (PGL) in association with cyanotic congenital heart disease (CHD), polycythemia and some additional tumors. Here, we present 3 cases of PGL with closely located EPAS1 mutations. Despite the common gene location, their manifestations are different. 1. A 14-year-old girl found at age 9 years (yrs) to have polycythemia (Hb 177-195 g/l) and hypertension. At age 14 years, she was diagnosed to have multiple mid abdomenal and the urinary bladder PGLs. U. noremetanephrine was 35.8 umol/day (0-3.43). No evidence of somatostatinoma so far. Family hx is negative for PGL and WES revealed no germline mutations in PGL-associated genes. Tumor testing revealed a novel heterozygous EPAS1 variant (c.1589C>T, p.A530V). 2. A 32-yr-old woman has a complex cyanotic CHD diagnosed since birth. She underwent five cardiac surgeries between 1993 and 2015. In 2011, she was diagnosed with two abdominal PGLs. She underwent surgery and remained free of disease until 2019 when she developed recurrence with metastases (mets) to the liver and spine. She received external beam radiotherapy to the spine. Currently PGL mets remained stable. WES was negative for any germline mutation but tumor testing revealed a heterozygous EPAS1 mutation (c.1591C>T, p.P531S). 3. A 55-yr-old man diagnosed to have an upper abdominal PGL with lung and bone metastases at the age of 9 yrs. Biopsy from a right femoral met confirmed the diagnosis of bone mets. He underwent four abdominal surgeries between 1978-2008 and received MIBG Tx 6 times with a cumulative dose is 586 mCi. His last surgery in 2008 revealed PGLs but also a non-functioning Pancreatic neuroendocrine tumor (pNET). PGL mets remained stable over the last 15 yrs. WES revealed no germline mutation in any of the known PGL genes but tumor testing revealed an EPAS1 mutation (c.1592C>A, p.P531H). Discussion:EPAS1 mutations are very rare. In this series, we present 3 pts with EPAS1 mutations in 3 adjacent nucleotides (c.1589, 1591, 1592) in 2 adjacent codons (p.530-531). These mutations are located in a domain that is important for recognition by propyl hydroxylases that normally lead to inactivation and proteasomal degradation of HIF2a (EPAS1). Despite the common location, the manifestations are quite different. Although all of them developed PGL at a young age, Pt # 1 had severe polycythemia, Pt#2 had complex CHD, and pt#3 had pNET. The first two patients had extensive mets but these mets have a smoldering course, especially in pt#3 who has been living with these mets for 44 yrs! Since EPAS1 mutations are usually postzygotic, as suggested by its involvement of more than one organ, it is possible that the variable presentations of these same domain adjacently located mutations is related to the timing of their post zygotic development. Another possibility is that the variable expression of the mutated EPAS1 in different tissues Presentation: Saturday, June 17, 2023

Two successful pregnancies in a patient with Fontan-repaired dextrocardiac univentricular heart

Univentricular dextrocardia is a rare complex congenital cyanotic heart disease. With the improvement of medical and surgical care, a greater number of women with complex congenital heart diseases have survived up to the childbearing age. Pregnancy with univentricular dextrocardia and Fontan circulation increases maternal and fetal mortality and morbidity. We report a case of successful management of two pregnancies in a woman with Fontan repaired dextrocardiac univentricular heart.

Epidemiological characteristics and trends in postoperative death in children with congenital heart disease (CHD): a single-center retrospective study from 2005 to 2020

ObjectivesTo analyze the epidemiological characteristics and trends in death after thoracotomy in children with congenital heart disease (CHD).MethodsThe clinical data of children with CHD aged 0–14 years who died after thoracotomy in our hospital from January 1, 2005, to December 31, 2020, were retrospectively collected to analyze the characteristics of and trends in postoperative death.ResultsA total of 502 patients (365 males; 72.7%) died from January 1, 2005, to December 31, 2020, with an average of 31 deaths per year. For these patients, the median age was 2.0 months, the median length of hospital stay was 16.0 days, the median postoperative time to death was 5.0 days, and the median risk adjustment in congenital heart surgery-1 (RACHS-1) score was 3.0. 29.5% underwent emergency surgery, 16.9% had postoperative ECMO support, and 15.9% received postoperative blood purification treatment. In the past 16 years, the deaths of children with CHD under 1 year old accounted for 80.5% of all deaths among children with CHD aged 0–14 years, and deaths (349 cases) under 6 kg accounted for 69.5% of all deaths. Age at death, weight, and disease type were characterized by annual changes.ConclusionsThe postoperative deaths of children with CHD mainly occurred in infants and toddlers who weighed less than 6.0 kg, and TGA and PA were the most lethal CHDs. The proportion of deaths has been increasing across the years among patients who are young, have a low body weight, and have complex cyanotic CHD.

Coexistence of proboscis lateralis and multiple craniofacial, neurological, cardiac and spinal deformities: a one-of-a-kind case report

BackgroundProboscis lateralis (PL) is an uncommon congenital facial deformity marked by the protrusion of a primitive tubular structure made up of skin and soft tissue that generally emerges from the eye's medial canthus and is associated with some craniofacial deformities. We report the first case of PL with multiple craniofacial, neurological, cardiac, and spinal anomalies.Case presentationA full-term female baby delivered by cesarean section cried immediately at birth. The mother reported having a normal pregnancy but has a history of x-ray during her first trimester. The baby was born with a rare presentation of proboscis lateralis which was accompanied by multiple anomalies, including but not limited to bilateral colpocephaly, corpus callosum agenesis, complex cyanotic congenital heart disease, and hemivertebra of the T10 body.ConclusionPL is an uncommon congenital condition that causes a variety of craniofacial abnormalities. Multiple additional defects affecting various organ systems should also be evaluated in a person diagnosed with PL.

Altered white matter connectivity in children with congenital heart disease with single ventricle physiology

Children born with congenital heart disease (CHD) have seen a dramatic decrease in mortality thanks to surgical innovations. However, there are numerous risk factors associated with CHD that can disrupt neurodevelopment. Recent studies have found that psychological deficits and structural brain abnormalities persist into adulthood. The goal of the current study was to investigate white matter connectivity in early school-age children (6–11 years), born with complex cyanotic CHD (single ventricle physiology), who have undergone Fontan palliation, compared to a group of heart-healthy, typically developing controls (TPC). Additionally, we investigated associations between white matter tract connectivity and measures on a comprehensive neuropsychological battery within each group. Our results suggest CHD patients exhibit widespread decreases in white matter connectivity, and the extent of these decreases is related to performance in several cognitive domains. Analysis of network topology showed that hub distribution was more extensive and bilateral in the TPC group. Our results are consistent with previous studies suggesting perinatal ischemia leads to white matter lesions and delayed maturation.

A rare association of complex cyanotic congenital heart disease with situs inversus totalis complicated by brain abscess in an adolescent – A case report

A rare association of complex cyanotic congenital heart disease with situs inversus totalis complicated by brain abscess in an adolescent – A case report

Optical coherence tomography of the pulmonary arteries in children with congenital heart diseases: A systematic review.

Optical coherence tomography (OCT) is a high-resolution intravascular imaging tool and has shown promise for providing real-time quantitative and qualitative descriptions of pulmonary vascular structures in vivo in adult pulmonary hypertension (PH), while not popular in pediatric patients with congenital heart diseases (CHD). The aim of this review is to summarize all the available evidence on the use of OCT for imaging pulmonary vascular remodeling in pediatric patients. We conducted the systematic literature resources (Cochran Library database, Medline via PubMed, EMBASE, and Web of Knowledge) from January 2010 to December 2021 and the search terms were "PH", "child", "children", "pediatric", "OCT", "CHD", "pulmonary vessels", "pulmonary artery wall". Studies in which OCT was used to image the pulmonary vessels in pediatric patients with CHD were considered for inclusion. Five studies met the inclusion criteria. These five papers discussed the study of OCT in the pulmonary vasculature of different types of CHD, including common simple CHD, complex cyanotic CHD, and Williams-Beuren syndrome. In biventricular anatomy, pulmonary vascular remodeling was primarily reflected by pulmonary intima thickening from two-dimensional OCT. In single-ventricle anatomy, due to the state of hypoxia, the morphology of pulmonary vessels was indirectly reflected by the number and shape of nourishing vessels from three-dimensional OCT. OCT may be an adequate imaging procedure for the demonstration of pulmonary vascular structures and provide additional information in pediatric patients.

Neointimal hyperplasia in systemic-to-pulmonary shunts of children with complex cyanotic congenital heart disease.

Neointimal hyperplasia might affect systemic-to-pulmonary shunt failure in infants with complex cyanotic congenital heart disease. The aim of this study was to elucidate histopathologic changes in polytetrafluoroethylene shunts and to determine whether increased neointimal formation is associated with early interventions comprising balloon dilatation, stent implantation and shunt revision. Furthermore, we intended to identify clinical factors associated with increased neointimal proliferation. Removed shunts were processed for histopathological analysis. Slides were stained with hematoxylin/eosin and Richardson. Immunohistochemistry was performed with anti-alpha-smooth muscle actin and anti-CD68. Non-parametric analysis and univariable regressions were performed to identify clinical factors associated with neointimal hyperplasia and shunt stenosis. Fifty-seven shunts (39 modified Blalock-Taussig anastomosis, 8 right ventricle-to-pulmonary artery anastomosis, 10 central shunts) were analysed. Area of neointimal proliferation within the shunt was in median 0.75 mm2 (interquartile range, 0.3-1.57 mm2) and relative shunt stenosis in median 16.7% (interquartile range, 6.7-30.8%). Neointimal hyperplasia and shunt stenosis correlated with each other and were significantly greater in the group that required early interventions and shunt revision. Univariable linear regression identified smaller shunt size and lower acetylsalicylic acid dosage as factors to be associated with greater neointimal proliferation and shunt stenosis. In infants with complex cyanotic congenital heart disease, neointimal hyperplasia in systemic-to-pulmonary shunts is associated with early interventions comprising balloon dilatation, stent implantation and shunt revision. Smaller shunt size and lower aspirin dosage are associated with increased neointimal proliferation.

Rastelli procedure in transposition of the great arteries associated with ventricular septal defect and pulmonary stenosis: A report of 3 consecutive cases in Cho Ray hospital

Transposition of the great arteries (TGA) is a complex cyanotic congenital heart disease, and only 10% of children with TGA can be alive through infancy. Rastelli operation is a standard surgical method for patient with TGA associated with ventricular septal defect (VSD) and pulmonary stenosis (PS). The aim of this report was to show short-term outcome of Rastelli operation in TGA; 3 patients (aged from 3 months to 4 years) were diagnosed TGA, VSD, atrial septal defect (ASD) and PS (2 severe pulmonary stenosis, 1 pulmonary atresia) with cyanosis, failure to thrive, baseline oxygen saturation values of 62% to 75%. All of 3 was performed Rastelli procedure and hospital discharged after 14 – 17 days postoperatively with saturation > 95%, no early complication, reoperation or death. Rastelli procedure in TGA associated with VSD, ASD and PS is initially well performed at the department of Pediatric Cardiac Surgery, Cho Ray hospital with satisfactory short-time outcome

Experience in Performing Bidirectional Cavopulmonary Anastomosis in the Surgical Treatment of Tricuspid Atresia

Tricuspid atresia (TA) is a rare cyanotic congenital heart disease (CHD) first described by Kreysig (1817). Bidirectional cavopulmonary anastomosis (BCPA) is part of the hemodynamic correction of such a complex combined CHD. In patients after BCPA, blood flow through the superior vena cava, providing efficient pulmonary blood flow, is the most important factor influencing blood oxygen saturation.
 The aim. To evaluate perioperative characteristics of the patients, immediate and long-term results after BCPA with TA.
 Materials and methods. In the period from January 1996 to April 2022, 50 patients with TA and multiple concomitant CHD underwent hemodynamic correction at the National Amosov Institute of Cardiovascular Surgery of the National Academy of Medical Sciences of Ukraine. There were 27 male patients (54%) and 23 female patients (46%). The mean age of the patients at the time of surgery was 31.6 ± 17.2 months (from 6 to 144 months). The main method of diagnosis in determining the defect and assessing the immediate and long-term results was echocardiographic examination and probing of the heart cavities. Among the 50 patients surveyed, 17 children were older than 2.5 years, so all the subjects were divided into 2 age groups: group I (n = 33) up to 2.5 years, group II (n = 17) older than 2.5 years to determine the optimal time of hemodynamic correction based on immediate and long-term results.
 Results. In the early postoperative period, there were 2 (4%) deaths among the patients of group II. The cause of death was acute heart failure in one case and infectious factor which led to the development of sepsis in another. There was a very interesting trend of increase in the duration of the operation (min): 239 ± 73.3 in group I, 251 ± 68.5 in group II; duration of mechanical ventilation (hours): 5.3 ± 1.4 in group I, 11 ± 3.7 in group II; duration of exudation (hours): 67 ± 22.9 in group I, 76 ± 26.8 in group II. There was significantly higher average dose of sympathomimetic (μg/kg/hour): 5 ± 2.1 for 60 ± 21.4 (hours) in group I; 7 ± 4.5 for 84 ± 29 (hours) in group II, hospital stay (days): 18 ± 5.7 in group I, 22 ± 11.3 in group II. Significantly lower mean systemic saturation at discharge 81 ± 4.6 (%) which was reported in children of group II compared to those of group I 88 ± 3.4 (%) clearly shows a decrease in the contribution of superior vena cava flow into the systemic circulation depending on the patient’s age, therefore, the clinical effect of performing BCPA as a stage of single-ventricular correction in patients with TA is much better when performing surgery in early childhood. Early postoperative period was uneventful in 27 (54%) patients. The other 23 (46%) patients of both groups had 16 and 18 complications in 10 (20%) patients of group I and 13 (26%) patients of group II, respectively, due to adverse factors of preoperative condition and specific changes in blood circulation characteristic of the BCPA. During the observation period lasting 49 ± 23.5 months, from 6 to 180 months, the final stage of hemodynamic correction was examined and performed: total cavopulmonary anastomosis was created in 31 (65%) patients. No deaths were reported after the final stage of hemodynamic correction.
 Conclusions. The contribution of superior vena cava flow to total cardiac output is directly related to the patient’s age and gradually decreases in the elderly, which indicates a decrease in systemic saturation, so the clinical effect of BCPA as a stage of single ventricular correction in patients with TA is much better when performing surgery in early childhood.

Complex cyanotic congenital heart disease presenting as congenital heart block in a Nigerian infant: case report and literature review

Background: The prevalence of cyanotic congenital heart diseases (CCHD) varies world wide. It accounts for a third of all congenital heart diseases. The common CCHD includes Tetralogy of Fallot(TOF), transposition of the great arteries (TGA), total anomalous pulmonary venous return (TAPVR), truncus arteriosus, and tricuspid atresia (TA). Less common variants include Ebstein’s anomaly, Hypoplastic left heart syndrome, pulmonary atresia, and single ventricle. Children of all ages can be are affected. The commonest presentation is cyanosis. Bradycardia and/or congenital heart block are rare presentations and mostly occur in the presence of an associated congenital atrioventricular block. Case report: We report a case of a 3-month-old female presenting with congenital heart block and bradycardia at 3 months of age and found to have complex cyanotic congenital heart disease on echocardiography. Conclusion: An infant presenting with bradycardia clinically should be screened for congenital heart defect as bradycardia may be an ominous sign of serious underlying cardiac defect.

Computed tomography in tetralogy of Fallot: pre- and postoperative imaging evaluation.

Tetralogy of Fallot (TOF) is the most common cause of cyanotic congenital heart disease (CHD) and the most frequent complex CHD encountered in adulthood. Although children with TOF share four characteristic features (subaortic ventricular septal defect, overriding aorta, right ventricular hypertrophy, pulmonary stenosis), the clinical spectrum and course are in fact greatly heterogeneous. Echocardiography remains the mainstay for diagnosis, presurgical planning and postoperative follow-up. However, with continued technological advances, CT now plays an increasing role in TOF evaluation and management, helping to minimize routine invasive catheter angiography. Preoperatively, CT is uniquely suited to assess associated pulmonary arterial, aortic and coronary anomalies as well as extra-cardiovascular structures and is particularly helpful for delineating complex anatomy in the TOF subtypes of absent pulmonary valve and pulmonary atresia with major aortopulmonary collaterals. Postoperatively, CT is useful for identifying surgical complications and for long-term monitoring including volumetry quantification, especially in children for whom MRI is contraindicated or limited by implanted devices such as pacemakers and stents. In this article, we review key clinical features and considerations in the pre- and postoperative TOF patient and the burgeoning role of CT for facilitating accurate diagnosis and personalized intervention.

Commentary: The bidirectional Glenn procedure can be useful in palliating late-presenting patients with complex cyanotic congenital heart disease.

Journal of Cardiac SurgeryVolume 36, Issue 9 p. 3215-3216 COMMENTARY Commentary: The bidirectional Glenn procedure can be useful in palliating late-presenting patients with complex cyanotic congenital heart disease John J. Lamberti MD, Corresponding Author John J. Lamberti MD jlamberti@stanford.edu orcid.org/0000-0002-7182-7414 Division of Pediatric Cardiac Surgery, Department of Cardiothoracic Surgery, Stanford University and Lucile Packard Children's Hospital, Falk Cardiovascular Research Center, Stanford, California, USA Correspondence John J. Lamberti, MD, Division of Pediatric Cardiac Surgery, Department of Cardiothoracic Surgery, Stanford University and Lucile Packard Children's Hospital, 300 Pasteur Dr., Falk Cardiovascular Research Center, Stanford, CA 94305-5407, USA. Email: jlamberti@stanford.eduSearch for more papers by this author John J. Lamberti MD, Corresponding Author John J. Lamberti MD jlamberti@stanford.edu orcid.org/0000-0002-7182-7414 Division of Pediatric Cardiac Surgery, Department of Cardiothoracic Surgery, Stanford University and Lucile Packard Children's Hospital, Falk Cardiovascular Research Center, Stanford, California, USA Correspondence John J. Lamberti, MD, Division of Pediatric Cardiac Surgery, Department of Cardiothoracic Surgery, Stanford University and Lucile Packard Children's Hospital, 300 Pasteur Dr., Falk Cardiovascular Research Center, Stanford, CA 94305-5407, USA. Email: jlamberti@stanford.eduSearch for more papers by this author First published: 06 June 2021 https://doi.org/10.1111/jocs.15723Citations: 1Read the full textAboutPDF ToolsRequest permissionExport citationAdd to favoritesTrack citation ShareShare Give accessShare full text accessShare full-text accessPlease review our Terms and Conditions of Use and check box below to share full-text version of article.I have read and accept the Wiley Online Library Terms and Conditions of UseShareable LinkUse the link below to share a full-text version of this article with your friends and colleagues. Learn more.Copy URL Share a linkShare onFacebookTwitterLinked InRedditWechat No abstract is available for this article.Citing Literature Volume36, Issue9September 2021Pages 3215-3216 RelatedInformation

A Rare Combination of Complex Congenital Heart Disease with Acquired Rheumatic Valvular Heart Disease

Tetralogy of Fallot (TOF) is a complex cyanotic congenital heart disease with a survival beyond middle age. Rheumatic and congenital cyanotic heart disease are common causes of hospital admission. However, coexistence of rheumatic heart disease with complex congenital heart disease like TOF is known to occur very rarely. This report presents a case of rheumatic valvular heart disease (AR & MR) with a complex congenital heart disease (TOF with PDA with PLSVC) in a 30 yrs old female. Patient was treated by conservative management and advised for corrective surgery and double valve replacement.
 Cardiovasc. j. 2021; 13(2): 235-238

Liver-only Transplantation in a Patient With Complex Congenital Heart Disease: Case Report and Review of the Literature.

Background.Patients with liver failure due to or in addition to congenital heart disease (CHD) represent a growing population in need of organ transplantation. Traditionally, these patients received a combined heart and liver transplantation carrying a high risk of perioperative morbidity and mortality.Methods.We discuss a patient with complex cyanotic CHD and biliary atresia undergoing liver-only transplantation. Furthermore, a literature study was performed on combined congenital heart and liver disease in the setting of transplantation.Results.We describe a unique case of a patient with severe CHD undergoing orthotopic liver transplantation for biliary atresia. In the literature, congenital malformations affecting different organs seems not that infrequent. Liver-only transplantation has been described in mild CHD, although data in adult patients are scarce. In severe CHD, the liver usually suffers from congestion. The severity of liver disease and reversibility should be estimated to decide on combined heart-liver transplantation.Conclusions.Our case and a review of the literature demonstrate that a patient-tailored approach with liver-only transplantation may be an appropriate alternative to combined heart and liver transplantation in selected cases.

Value of Ultrasonic Cardiogram Combined with Flash CT in Diagnosis of Conotruncal Defects

The paper aims at exploring the value of ultrasonic cardiogram (UCG) combined with Flash CT in the diagnosis of conotruncal defects (CTD). This complex cyanotic congenital heart disease is divided into several types by using UCG and Flash dual source CT imaging. The results show that the detection method of UCG is simple and fast, but it has some deficiencies in the display of extracardiac vessels. Flash CT technology has a strong image post-processing function, which makes up for the shortcomings of UCG and is an important supplementary means for patients after echocardiographic examination. Therefore, UCG combined with Flash CT technology can detect the patient's disease more accurately and quickly, avoid the traumatic detection of angiography, and is more convenient. It is hopeful to replace angiography as the gold standard for the diagnosis of congenital heart disease.

Current era outcomes of pulmonary atresia with ventricular septal defect: A single center cohort in Thailand

Pulmonary atresia with ventricular septal defect (PA/VSD) is a complex cyanotic congenital heart disease with a wide-range of presentations and treatment strategies, depending on the source of pulmonary circulation, anatomy of pulmonary arteries (PAs), and major aortopulmonary collateral arteries (MAPCAs). Data about the outcomes in developing countries is scarce. We therefore conducted a retrospective study to assess survival rates and mortality risks of 90 children with PA/VSD at Siriraj Hospital, Thailand during 2005–2016. Patients with single ventricle were excluded. Survival and mortality risks were analyzed at the end of 2018. The median age of diagnosis was 0.5 (0–13.8) years. The patients’ PAs were categorized into four groups: 1) PA/VSD with confluent PAs (n = 40), 2) PA/VSD with confluent PAs and MAPCAs (n = 21), 3) PA/VSD with non-confluent PAs and MAPCAs (n = 12), and 4) PA/VSD with small native PAs and MAPCAs (n = 17). Of the 88 patients who underwent operations, 32 patients had complete repair at 8.4 ± 4.6 years old. During the follow-up [median time of 5.7 years (7 days-13.6 years)], 17 patients (18.9%) died. The survival rates at 1, 5, and 10 years of age were 95%, 83.7%, and 79.6%, respectively. Significant mortality risks were the presence of associated anomalies and non-confluent PAs.

PULMONARY ARTERY CATHETERIZATION VIA FEMORAL ARTERY ACCESS

Unoperated pulmonary atresia with ventricular septal defect (VSD) is a complex cyanotic congenital heart disease rarely compatible with survival to adulthood. A 34-year-old Palestinian female with a reported history of Tetralogy of Fallot (TOF) presents with worsening fatigue. Vital signs are