Complex Congenital Cardiac Anomalies Research Articles

ACUTE RESPIRATORY DISTRESS REVEALING A COMPLETE ATRIOVENTRICULAR CANAL IN A 22-MONTH INFANT

The atrioventricular canal (AVC) is a complex and rare congenital cardiac anomaly. It is potentially severe due to the complexity of its lesions and associated fixed pulmonary arterial hypertension. We report the case of a 22-month-old infant girl with Trisomy 21 syndrome, admitted for acute respiratory distress. TTE showed a complete AVC with pulmonary arterial hypertension. Prognosis depends on the promptness of diagnosis and therapeutic management.

Outcomes and Trends of Surgical Ventricular Septal Defect Repair in a Tertiary Care Hospital in Pakistan: a Retrospective Study

Background: Ventricular septal defect is the most common congenital heart malformation in newborns. About 77% of newborns diagnosed with congenital heart disease had ventricular septal defect in Pakistan. VSD closure is one of the commonest procedure performed by pediatric cardiac surgeons. With advancement in preoperative and postoperative management, VSD closure has a very low incidence of postoperative complications. Methodology: Patients who underwent VSD closure at our center from 2017 to 2021 were reviewed in the database. Patients with a simple VSD were included and those with a concomitant complex congenital cardiac anomaly were excluded. Data was analyzed in SPSS 23. Results: Among the patients, 62.8% were males and 37.2% were females. Mean age was 10.36 + 9.69 years, with 39.7% of the subjects below the age of 5. Increased ICU stay was defined as stay in the ICU for more than 48 hours and increased hospital stay was described as postoperative stay of more than 7 days. Nine patients had a prolonged hospital stay and four patients had a prolonged ICU stay. One patient went under reopening due to bleeding tamponade and one had a deep sternum infection. In hospital mortality rate was 2.4%. Conclusion: VSD has a very low mortality rate in our center. Further studies are required to identify risk factors for morbidity and mortality. Long term follow-up studies should be conducted to identify rate of long term complications.

Adverse Events in the Pediatric Cardiac Catheterization Suite: Does Having Pediatric Cardiac Anesthesiologists Make a Difference?

It has been suggested that anesthesiologists with subspecialty expertise in pediatric cardiac anesthesia are best qualified to care for patients with complex congenital cardiac anomalies and manage the complex physiology frequently encountered in the pediatric cardiac catheterization lab. We evaluated the incidence of adverse events in our pediatric cardiac catheterization lab, comparing care provided by cardiac and noncardiac pediatric attending anesthesiologists. Data were collected on each anesthetic in the pediatric cardiac catheterization lab from January 1, 2016 to December 31, 2019. A generalized linear mixed effect model was used to identify associations between pediatric cardiac and noncardiac anesthesiologists and the presence of adverse events adjusting for age, American Society of Anesthesiologists physical status, emergency status, and interventional versus diagnostic procedures. A total of 3,761 procedures involving 1,729 patients were included in the study. There was no significant difference between noncardiac and cardiac anesthesia attendings for overall adverse events (odds ratio [OR], 1.2; 95% confidence interval [CI], 0.82 to 1.75 P=0.349). Specific respiratory adverse events (OR, 1.22; 95%, CI 0.73 to 2.03 P=0.443) or cardiac adverse events (OR, 1.26; 95% CI, 0.64 to 2.48 P=0.502) were also not significantly different with respect to noncardiac compared with cardiac attending anesthesiologists. In our analysis, the incidence of adverse events in the pediatric cardiac catheterization lab during the study period was not statistically different, whether anesthesia care was provided by a cardiac or a noncardiac anesthesiologist.

УСПЕШНАЯ КОРРЕКЦИЯ ОБЩЕГО АТРИОВЕНТРИКУЛЯРНОГО КАНАЛА С ТЕТРАДОЙ ФАЛЛО И ГИПОПЛАЗИЕЙ ПРАВОГО ЖЕЛУДОЧКА У РЕБЕНКА 2 ЛЕТ 4 МЕСЯЦЕВ

The paper presents a description of a case of successful one and a half ventricular correction of a complex congenital cardiac anomalies, including the common atrioventricular canal, tetralogy of Fallot, and hypoplasia of the right ventricle in a child (2 year and 4 months). Ararecongenitalanomalycharacterized by acombinationof intracardiac defects, required an innovative surgical approach, the exclusivity of this clinical case has determined. The surgical strategy of complete atrioventricular canal defect is determined by a number of factors. One of the most important is balance between right and left ventricular outputs. The balanced type with adequate development of the ventricles involves the biventricular repair performance. Unbalancedatrioventricular canal defects includea hypoplastic ventricle. Reconstructive surgeryfor onehypoplasticventricle is oriented towards the degree of hypoplasia. In particular, a mild right ventricular hypoplasia allows perfoming biventricular radical operation while a severe degree suggests univentricular repair. In case of borderline, moderate degree of hypoplastic right ventricle the one and half ventricle repair can be carried out. Another important point is the presence of concomitant pulmonic stenosis required the choice of optimal method and material for reconstruction which is not always obvious and often it is the subject of debates. This report presents a description of the diagnostic stages with an emphasis on determining the type of balance of the common atrioventricular canal, the degree of hypoplasia of the right ventricle, the approach to choosing the optimal method for correcting the defect in general and the material for reconstructing the outflow pathway from the right ventricle in particular, as well as the subsequent successful correction of congenital multicomponent cardiac abnormalities in a young patient.

Coronary artery-pulmonary artery fistula in pulmonary atresia with ventricular septal defect: report of two surgical cases.

Pulmonary atresia with ventricular septal defect is a complex congenital cardiac anomaly. The blood is supplied to the lungs through a patent ductus arteriosus, a major aortopulmonary collateral artery, or in very rare cases from a coronary artery-pulmonary artery fistula. We present two cases with coronary artery-pulmonary artery fistula which underwent surgical intervention. In our first patient, the main pulmonary artery was supplied from the left main coronary artery. In the second patient, the right pulmonary artery originated from the left main coronary artery and continued to the right lung posteriorly to the aorta, while the left pulmonary artery originated from the patent ductus arteriosus. The difference in our cases is that the coronary artery pulmonary artery fistulas behave like major aortopulmonary collateral arteries originating from the coronary arteries. These fistulas were the main source of pulmonary blood flow.

Vasoplegic syndrome after cardiovascular surgery: A review of pathophysiology and outcome-oriented therapeutic management.

Vasoplegic syndrome (VPS) is defined as systemic hypotension due to profound vasodilatation and loss of systemic vascular resistance (SVR), despite normal or increased cardiac index, and characterized by inadequate response to standard doses of vasopressors, and increased morbidity and mortality. It occurs in 9%-44% of cardiac surgery patients after cardiopulmonary bypass (CPB). The underlying pathophysiology following CPB consists of resistance to vasopressors (inactivation of Ca2+ voltage gated channels) on the one hand and excessive activation of vasodilators (SIRS, iNOS, and low AVP) on the other. Use of angiotensin-converting enzyme inhibitor (ACE-I), calcium channel blockers, amiodarone, heparin, low cardiac reserve (EF < 35%), symptomatic congestive heart failure, and diabetes mellitus are the perioperative risk factors for VPS after cardiac surgery in adults. Till date, there is no consensus about the outcome-oriented therapeutic management of VPS. Vasopressors such as norepinephrine (NE; 0.025-0.2 µg/kg/min) and vasopressin (0.06 U/min or 6 U/h median dose) are the first choice for the treatment. The adjuvant therapy (hydrocortisone, calcium, vitamin C, and thiamine) and rescue therapy (methylene blue [MB] and hydroxocobalamin) are also considered when perfusion goals (meanarterial pressure [MAP] > 60-70 mmHg) are not achieved with nor-epinephrine and/or vasopressin. The aims of this systematic review are to collect all the clinically relevant data to describe the VPS, its potential risk factors, pathophysiology after CPB, and to assess the efficacy, safety, and outcome of the therapeutic management with catecholamine and non-catecholamine vasopressors employed for refractory vasoplegia after cardiac surgery. Also, to elucidate the current and practical approach for management of VPS after cardiac surgery. "PubMed," "Google," and "Medline" weresearched, and over 150 recent relevant articles including RCTs, clinical studies, meta-analysis, reviews, case reports, case series and Cochrane data were analyzed for this systematic review. The filter was applied specificallyusing key words like VPS after cardiac surgery, perioperative VPS following CPB, morbidity, and mortality in VPS after cardiac surgery, vasopressors for VPS that improve outcomes, VPS after valve surgery, VPS after CABG surgery, VPS following complex congenital cardiac anomalies corrective surgery, rescue therapy for VPS, adjuvant therapy for VPS, definition of VPS, outcome in VPS after cardiac surgery, etiopathology of VPS following CPB. This review did not require any ethical approval or consent from the patients. Despite the recent advances in therapy, the mortality remains as high as 30%-50%. NE has been recommended the most frequent used vasopressor for VPS. It restores and maintain the MAP and provides the outcome benefits. Vasopressin rescue therapy is an alternative approach, if catecholamines and fluid infusions fail to improve hemodynamics. It effectively increases vascular tone and lowers CO, and significantly decreases the 30 days mortality. Hence, suggested a first-line vasopressor agent in postcardiac surgery VPS. Terlipressin (1.3μg/kg/h), a longer acting and more specific vasoconstrictor prevents the development of VPS after CPB in patients treated with ACE-I. MB significantly reduces morbidity and mortality of VPS. The Preoperative MB (1%, 2mg/kg/30min, 1h before surgery) administration in high risk (on ACE-I) patients for VPS undergoing CABG surgery, provides 100% protection against VPS, and early of MB significantly reduces operative mortality, and recommended as a rescue therapy for VPS. Hydroxocobalamin (5 g) has been recommended as a rescue agent in VPS refractory to multiple vasopressors. A combination of ascorbic acid (6 g), hydrocortisone (200 mg/day), and thiamine (400 mg/day) as an adjuvant therapy significantly reduces the vasopressors requirement, and provides mortality and morbidity benefits. Currently, the VPS is frequently encountered (9%-40%) in cardiac surgical patients with predisposing patient-specific risk factors and combined with inflammatory response to CPB. Multidrug therapy (NE, MB, AVP, ATII, terlipressin, hydroxocobalamin) targeting multiple receptor systems is recommended in refractory VPS. A combination of high dosage of ascorbic acid, hydrocortisone and thiamine has been used successfully as adjunctive therapyto restore the MAP. We also advocate for the early use of multiagent vasopressors therapy and catecholamine sparing adjunctive agents to restore the systemic perfusion pressure with a goal of preventing the progressive refractory VPS.

Ventricular septal defect with pulmonary atresia: approaches, results, prognosticators and current status.

Ventricular septal defect with pulmonary atresia and major aortopulmonary collateral arteries is a complex congenital cardiac anomaly with a wide spectrum of anatomical variations. Akin to the same, the management options are also very diverse ranging from aggressive single-stage repair with unifocalisation to surgical palliation and/or staged repair and also heart transplant. There is no consensus on the best management option. This review aims at highlighting the various surgical options and proposing a management pathway suited for the subcontinent patients.

Extracorporeal Cardiopulmonary Resuscitation During Cesarean Delivery in a Patient With D-Transposition of the Great Vessels, Years Following Mustard Operation

Patients with surgically repaired complex congenital cardiac anomalies present unique characteristics that can make the implementation of extracorporeal membrane oxygenation (ECMO) support especially challenging. Very few series have reported the outcomes of ECMO support during pregnancy and peripartum. We report a case of successful extracorporeal cardiopulmonary resuscitation during cesarean delivery in a patient with surgically repaired d-transposition of the great arteries, and we discuss particular aspects that contributed to successful implementation of ECMO support and hospital discharge.

Complex congenital cardiac anomalies in the setting of right isomerism in a 31-month-old infant: a case report

BackgroundCongenital cardiac defects are not rare among neonates. Prompt assessment for life-threatening anomalies is essential for rapid management decisions and positive outcomes. Extracardiac anomalies can occur in congenital heart defects, and their presence increases morbidity and mortality in these neonates.Case presentationWe report a case of a 31- month-old infant black girl in Tanzania who presented with an on-and-off history of difficulty in breathing, easy fatigability, facial and lower-limb swelling, recurrent respiratory tract infections, and failure to thrive.ConclusionsManagement of patients with heterotaxy syndrome is complex and largely depends on specific anatomy of both cardiac and noncardiac lesions. Cardiac and noncardiac management must be tailored to individual anatomy, including prophylaxis against encapsulated organisms for asplenic patients.

Cor Triatriatum: A Review.

Cor triatriatum is a rare congenital cardiac anomaly defined by an abnormal septation within the atrium (left or right) leading to inflow obstruction to the respective ventricles. It exists either in isolated classical form or may be associated with simple to complex congenital cardiac anomalies. Several anatomical variants exist even in the classical form, and therefore, it may require multimodal diagnostic modalities to characterize and differentiate for better percutaneous interventional or surgical planning. It commonly presents in infancy but may remain undetected till death. Symptomatology typically mimics mitral and tricuspid stenosis in sinister and dexter varieties, respectively. However, features of systemic embolization, heart failure, atrial fibrillation, cyanosis, cardiac asthma, syncope, and sudden cardiac arrest have also been reported in the literature. Surgical correction under cardiopulmonary bypass is the preferred treatment. Nevertheless, balloon dilatation may be considered in anatomically compatible variants and in special circumstances, such as heart failure, pregnancy, or as a bridge to definitive treatment.

Clinical application of three-dimensional printing to the management of complex univentricular hearts with abnormal systemic or pulmonary venous drainage.

In recent years, three-dimensional printing has demonstrated reliable reproducibility of several organs including hearts with complex congenital cardiac anomalies. This represents the next step in advanced image processing and can be used to plan surgical repair. In this study, we describe three children with complex univentricular hearts and abnormal systemic or pulmonary venous drainage, in whom three-dimensional printed models based on CT data assisted with preoperative planning. For two children, after group discussion and examination of the models, a decision was made not to proceed with surgery. We extend the current clinical experience with three-dimensional printed modelling and discuss the benefits of such models in the setting of managing complex surgical problems in children with univentricular circulation and abnormal systemic or pulmonary venous drainage.

Abstract 17889: A Randomized Controlled Trial of Moderate Glucose Control After Complex Pediatric Cardiac Surgery

Introduction: Infants and children after cardiac surgery may develop hyper- or hypoglycemia, associating with higher nosocomial infection or neurological morbidities. Hypothesis: The hypothesis of this perspective randomized control study is moderate glucose control may improve clinical outcomes. Methods: We randomly assigned children(≤3 years of age) who were admitted to the pediatric cardiac intensive care unit (PICU) after cardiopulmonary bypass surgery into either moderate glucose control group (target blood glucose: 110-143mg per deciliter) or conventional glucose control group (target level below 200mg per deciliter). The primary outcome was the rate of nosocomial infection in PICU. Second outcomes include hospital mortality, duration of mechanical ventilation and PICU stay, and a composite morbidity variable included events of requiring extracorporeal membrane oxygenation, delayed sternal closure, dialysis-dependent renal failure and hypoglycemia. Results: A total of 593 patients underwent randomization: 293 to moderate glucose control group and 300 to conventional glucose control group. The baseline data were balanced between these two groups. Mean 72 hours time-weighted blood glucose average was lower in the moderate control group than in the conventional group (129.9±22.1 mg per deciliter vs. 138.8±28.6 mg per deciliter, p&lt;0.001). Although no statistically significance reached, there is a trend that nosocomial infection occurred less in moderate glucose group (17 (5.80%) vs. 29 (9.67%), P=0.054). Duration of mechanical ventilation was shorter in the moderate control group (18 (range, 4-300) hours vs 22 (range 3-771) hours, p=0.046). Hypoglycaemia (blood glucose ≤65mg per deciliter) which occurred in 7(2.39%) patients in the moderate group versus 8 (2.67%) in the conventional group, was statistically similar between groups. Other secondary outcomes did not differ significantly between groups. Conclusions: With moderate glucose control, although the major clinical outcomes did not change, this randomized control study showed shorter ventilation time after pediatric cardiac surgery, substantially benefit for patients with complex congenital cardiac anomalies.

A Contemporary, Single-Institutional Experience of Surgical Versus Expectant Management of Congenital Heart Disease in Trisomy 13 and 18 Patients

The objective of this study was to examine a large institutional experience of patients with trisomy 13 and trisomy 18 in the setting of comorbid congenital heart disease and present the outcomes of surgical versus expectant management. It is a retrospective single-institution cohort study. Institutional review board approved this study. Thirteen consecutive trisomy 18 patients and three consecutive trisomy 13 patients (sixteen patients in total) with comorbid congenital heart disease who were evaluated by our institution's Division of Cardiovascular Surgery between January 2008 and December 2013 were included in the study. The primary outcome measures evaluated were operative mortality (for patients who received surgical management), overall mortality (for patients who received expectant management), and total length of survival during follow-up. Of the thirteen trisomy 18 patients, seven underwent surgical management and six received expectant management. With surgical management, operative mortality was 29 %, and 80 % of patients were alive after a median follow-up of 116 days. With expectant management, 50 % of patients died before hospital discharge. Of the three patients with trisomy 13, one patient underwent surgical management and two received expectant management. The patient who received surgical management with complete repair was alive at last follow-up over 2 years after surgery; both patients managed expectantly died before hospital discharge. Trisomy 13 and trisomy 18 patients with comorbid congenital heart disease can undergo successful cardiac surgical intervention. In this population, we advocate that nearly all patients with cardiovascular indications for operative congenital heart disease intervention should be offered complete surgical repair over palliative approaches for moderately complex congenital cardiac anomalies.

Concomitant congenital heart defect repair and Nuss procedure for pectus excavatum

The combination of complex congenital cardiac anomalies and pectus excavatum represents a technical challenge. Most concomitant repairs have been performed in adult patients. We report the uncomplicated concomitant repair of double-outlet right ventricle with absent pulmonary valve syndrome and the Nuss procedure for pectus excavatum in a 3-year-old child.

Fontan operation for the Cantrell syndrome using a clamshell incision

A median sternotomy could be difficult for a child with ectopia cordis and complex congenital cardiac anomalies. We report a patient with ectopia cordis, functionally single ventricle and bilateral superior vena cava, who underwent a staged Fontan procedure through a clamshell incision and the sternothoracotomy approach.

Surgical Placement of Permanent Epicardial Pacing Systems in Very Low-Birth Weight Premature Neonates

Few studies have characterized the surgical outcomes following epicardial pacemaker placement in very low-birth weight infants with congenital complete heart block. This study was undertaken to review the surgical experience with this patient population based on data from a large multi-institutional registry. The Pediatric Cardiac Care Consortium (PCCC) multi-institutional database was retrospectively reviewed to identify premature, low-birth weight neonates that underwent surgical placement of an epicardial pacing system for heart block. We reviewed 179 patients with birth weights less than 1.5 kg that underwent a major operative procedure. Of these, 10 patients underwent surgical placement of an epicardial pacing system for heart block. Patients had heart block in otherwise structurally normal hearts (n = 6) or heart block associated with complex structural congenital cardiac anomalies (n = 4). There were no deaths directly related to the surgical placement of the epicardial pacing system. There were no immediate complications with either lead or generator placement. One generator pocket was revised three months following placement. Survival to discharge was 60%. The four deaths occurred at a mean of 11 days (range 1-45 days) following the procedure. Neonates born with prematurity and congenital heart block represent a challenging subset of patients with significant mortality. Generator pocket breakdown and infection have been considered barriers to optimal short- and long-term outcomes. Among cases in the PCCC, there were no deaths or major complications that could be attributed to permanent epicardial pacemaker placement. These data suggest that an aggressive surgical strategy may be justified.

Ventricular assist device outflow graft in congenitally corrected transposition of great arteries - a surgical challenge

Congenitally corrected transposition of the great arteries is a complex congenital cardiac anomaly with a wide spectrum of morphologic features and clinical profiles. Most patients are diagnosed late in their life, undergoes surgical repairs, eventually leading to systemic ventricular failure needing heart transplant or mechanical circulatory assistance. As, aorta is located anterior to and left of the PA (Transposition of great arteries), the outflow graft of ventricular assist device traverse across pulmonary artery to reach aorta which poses challenge during further surgical explorations.

High Prevalence of Complex Congenital Cardiac Anomalies Detected by Fetal Echocardiography in a Cohort of Saudi Women Referred for Prenatal Assessment

With the new advances in computer technology, fetal echocardiography (FE) is becoming an accurate reliable tool for prenatal diagnosis of congenital heart diseases (CHD) which affect the postnatal care and management plans. We retrospectively described FE studies of 50 Saudi women referred for prenatal assessment. The mean maternal age was 30.6 -/+ 4.4 years; mean gestational age during the study was 26.16 -/+ 4.2 weeks. 58% had consanguineous marriage, and 72% had positive family history of CHD. CHD was found in 28% of cases. Postnatal echocardiographic study confirmed the prenatal diagnosis. The most common referral reason was history of CHD (56%) followed by abnormal sonographic findings during routine assessment (32%). However, the prevalence of CHD was higher in the second group (37.5%) than the first one (28.6%).There was no significant difference between cases with normal FE and those with CHD as regard the referral reasons. The most frequent lesion detected was those with univentricular morphology (42.85%), followed by atrioventricular septal defect (21.4%) and ventricular septal defect (VSD) (14.3%). When we categorized the structural CHD according to severity, complex CHD constituted 78.57%, while significant lesions were 14.3%. Eight cases had dysrhythmia, none of them had CHD.

Laparoscopic Assessment of a Migrated Ventriculoperitoneal Shunt into an Inguinal Hernia

Placement of a ventriculoperitoneal (VP) shunt may increase intraabdominal pressure and lead to an abdominal or genitourinary complication. We report on a 2-month-old boy with complex congenital neurologic and cardiac anomalies who had a VP shunt migrate into the left inguinal hernia. This report demonstrates how a laparoscopic approach can be successfully used to reposition the VP shunt, identify a contralateral inguinal hernia, and repair both without any additional incisions.

Noninvasive evaluations of pulmonary circulation, cardiac function, and liver excreting rate at late follow-up following the modified Fontan operation

The purpose of this paper was to define the late postoperative pulmonary circulation and cardiac and liver functions after a modified Fontan operation by radionuclide studies. Eleven patients (5 with single ventricle and 6 with double outlet right ventricle) underwent a modified Fontan operation using a RA-PA anastomosis. These 11 patients ranging in age from 7 to 42 years (mean 17±10 years) were examined at 4.7–12.1 years (mean 8.6±2.4 years) postoperatively and compared with studies in 10 normal adults. Pulmonary circulation, cardiac function and liver excreting function were determined by intravenous administration of Tc-MAA, Tc-PYP, and Tc-PMT, respectively. No extrapulmonary radioactivity was detected by Tc-MAA lung perfusion in patients. Although the cardiac diastolic function of the patients remained normal, the systolic functions (EF=46.4±7.8%, 1/3EF=11.2±4.4%, PER=1.93±0.54/sec) of the patients were significantly decreased compared with those of normal controls (EF=54.7±4.7%, 1/3EF=21.7±2.1%, PER=2.91±0.4/sec), respectively. The mean level of MTT of the patients (9.4±2.7 minutes) was also significantly prolonged compared with that of normal controls (6.1±4.4 minutes). Some of these deteriorated parameters clearly related to higher age at operation and length of intervals since operation. These data suggest that the earlier surgical intervention by the Fontan operation is desired for the complex congenital cardiac anomalies.