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1.
Article in English | MEDLINE | ID: mdl-38752879

ABSTRACT

We describe a surgical technique for a half-turned truncal switch operation in a 5-year-old child with dextro-transposition of the great arteries (D-TGA), a ventricular septal defect, a left ventricular outflow tract obstruction and a complex coronary pattern. The benefit of the half-turned truncal switch is the creation of haemodynamically superior biventricular outflow tracts and the maximal use of an autologous pulmonary valve in the right ventricular outflow tract, thereby avoiding the right ventricular-pulmonary artery conduit.


Subject(s)
Arterial Switch Operation , Heart Septal Defects, Ventricular , Transposition of Great Vessels , Ventricular Outflow Obstruction , Humans , Transposition of Great Vessels/surgery , Ventricular Outflow Obstruction/surgery , Heart Septal Defects, Ventricular/surgery , Child, Preschool , Arterial Switch Operation/methods , Male , Abnormalities, Multiple/surgery , Cardiac Surgical Procedures/methods , Ventricular Outflow Obstruction, Left
2.
Article in English | MEDLINE | ID: mdl-38775126

ABSTRACT

The arterial switch operation has evolved to become the treatment of choice for transposition of the great arteries and is one of the greatest success stories in congenital heart surgery. The most crucial step of the operation is the coronary artery translocation; therefore, it is of paramount importance for surgeons to know every single detail about the morphology and spatial relationships of the coronary arteries and the roots of the great vessels. However, sometimes the surgeon may face unfavourable scenarios such as major commissural malalignment and anomalous coronary artery patterns and need to be prepared to carry out a successful coronary artery translocation. Herein, we demonstrate that the trapdoor technique is useful for transferring coronary arteries in a neonate with major commissural malalignment and unusual coronary anatomy during the arterial switch operation.


Subject(s)
Arterial Switch Operation , Coronary Vessels , Transposition of Great Vessels , Humans , Transposition of Great Vessels/surgery , Arterial Switch Operation/methods , Arterial Switch Operation/adverse effects , Infant, Newborn , Coronary Vessels/surgery , Coronary Vessels/diagnostic imaging , Coronary Vessel Anomalies/surgery , Coronary Vessel Anomalies/diagnosis , Male
3.
Card Electrophysiol Clin ; 16(2): 139-142, 2024 Jun.
Article in English | MEDLINE | ID: mdl-38749632

ABSTRACT

This case report discusses a 42-year-old male with dextro-transposition of the great arteries (D-TGA) status post Mustard repair and sick sinus syndrome status post dual-chamber pacemaker implant, who developed symptomatic superior vena cava (SVC) baffle stenosis. He was treated with a combined pacemaker extraction and subsequent SVC baffle stenting. The case highlights the complexities of treating SVC baffle stenosis in the presence of cardiac implantable devices and demonstrates the efficacy of this combined approach. Furthermore, the authors delve into the intricacies of D-TGA, its surgical history, and the long-term complications associated with atrial switch procedures.


Subject(s)
Device Removal , Pacemaker, Artificial , Stents , Transposition of Great Vessels , Humans , Transposition of Great Vessels/surgery , Male , Adult , Pacemaker, Artificial/adverse effects , Superior Vena Cava Syndrome/surgery , Superior Vena Cava Syndrome/etiology , Sick Sinus Syndrome/therapy , Sick Sinus Syndrome/surgery
4.
Eur J Cardiothorac Surg ; 65(4)2024 Mar 29.
Article in English | MEDLINE | ID: mdl-38597905

ABSTRACT

OBJECTIVES: This study aimed to evaluate the short-/mid-term outcome of patients with complex dextro (d)-/levo (l)-transposition of the great arteries (TGA), ventricular septal defect and left ventricular outflow tract obstructions. METHODS: A single-centre, retrospective review of all complex dextro-TGA (n = 85) and levo-TGA (n = 22) patients undergoing different surgeries [Arterial switch operation + left ventricular outflow tract obstruction-resection (ASO-R), half-turned truncal switch/Mair (HTTS), Nikaidoh and Rastelli] between May 1990 and September 2022 was performed. Groups were analysed using Kruskal-Wallis test with post hoc pairwise comparison and Kaplan-Meier time-to-event models. RESULTS: A total of 107 patients [ASO-R (n = 20), HTTS (n = 23), Nikaidoh (n = 21), Rastelli (n = 43)] were included, with a median age of 1.0 year (0.5-2.5) and surgical repair median follow-up was 3.8 years (0.3-10.5). Groups did not differ in respect to early postoperative complications/early mortality. Five-year overall survival curves were comparable: ASO-R 78.9% (53.2-91.5), HTTS 75.3% (46.8-89.9), Nikaidoh 85% (60.4-94.9) and Rastelli 83.9% (67.5-92.5), P = 0.9. Highest rates of right ventricular outflow tract (RVOT) reinterventions [33.3% and 32.6% (P = 0.04)] and reoperations [28.6% and 32.6% (P = 0.02)] occurred after Nikaidoh and Rastelli procedures. However, overall freedom from RVOT reinterventions and RVOT reoperations at 5 years did not differ statistically significantly between the groups (ASO-R, HTTS, Nikaidoh and Rastelli): 94.4% (66.6-99.2), 69.1% (25.4-90.5), 67.8% (34-86.9), 64.4% (44.6-78.7), P = 0.2, and 90.0% (65.6-97.4), 91% (50.8-98.7), 65.3% (32.0-85.3) and 67.0% (47.4-80.6), P = 0.3. CONCLUSIONS: Surgical repair of complex dextro-/levo-TGA can be performed with satisfying early/mid-term survival. RVOT reinterventions/reoperations were frequent, with highest rates after Nikaidoh and Rastelli procedures. Left ventricular outflow tract obstruction reoperations were rare with zero events after Nikaidoh and HTTS procedures.


Subject(s)
Arterial Switch Operation , Transposition of Great Vessels , Ventricular Outflow Obstruction, Left , Ventricular Outflow Obstruction , Humans , Infant , Transposition of Great Vessels/surgery , Ventricular Outflow Obstruction/surgery , Arterial Switch Operation/adverse effects , Arterial Switch Operation/methods , Arteries , Retrospective Studies , Treatment Outcome
5.
Catheter Cardiovasc Interv ; 103(6): 943-948, 2024 May.
Article in English | MEDLINE | ID: mdl-38577955

ABSTRACT

BACKGROUND: Unilateral pulmonary artery (PA) stenosis is common in the transposition of the great arteries (TGA) after arterial switch operation (ASO) but the effects on the right ventricle (RV) remain unclear. AIMS: To assess the effects of unilateral PA stenosis on RV afterload and function in pediatric patients with TGA-ASO. METHODS: In this retrospective study, eight TGA patients with unilateral PA stenosis underwent heart catheterization and cardiac magnetic resonance (CMR) imaging. RV pressures, RV afterload (arterial elastance [Ea]), PA compliance, RV contractility (end-systolic elastance [Ees]), RV-to-PA (RV-PA) coupling (Ees/Ea), and RV diastolic stiffness (end-diastolic elastance [Eed]) were analyzed and compared to normal values from the literature. RESULTS: In all TGA patients (mean age 12 ± 3 years), RV afterload (Ea) and RV pressures were increased whereas PA compliance was reduced. RV contractility (Ees) was decreased resulting in RV-PA uncoupling. RV diastolic stiffness (Eed) was increased. CMR-derived RV volumes, mass, and ejection fraction were preserved. CONCLUSION: Unilateral PA stenosis results in an increased RV afterload in TGA patients after ASO. RV remodeling and function remain within normal limits when analyzed by CMR but RV pressure-volume loop analysis shows impaired RV diastolic stiffness and RV contractility leading to RV-PA uncoupling.


Subject(s)
Arterial Switch Operation , Cardiac Catheterization , Pulmonary Artery , Stenosis, Pulmonary Artery , Transposition of Great Vessels , Ventricular Function, Right , Adolescent , Child , Female , Humans , Male , Arterial Switch Operation/adverse effects , Compliance , Myocardial Contraction , Pulmonary Artery/physiopathology , Pulmonary Artery/diagnostic imaging , Retrospective Studies , Stenosis, Pulmonary Artery/physiopathology , Stenosis, Pulmonary Artery/diagnostic imaging , Stenosis, Pulmonary Artery/etiology , Stroke Volume , Transposition of Great Vessels/physiopathology , Transposition of Great Vessels/surgery , Transposition of Great Vessels/complications , Transposition of Great Vessels/diagnostic imaging , Treatment Outcome , Vascular Stiffness , Ventricular Dysfunction, Right/physiopathology , Ventricular Dysfunction, Right/etiology , Ventricular Dysfunction, Right/diagnostic imaging , Ventricular Pressure
6.
Genome Med ; 16(1): 53, 2024 Apr 03.
Article in English | MEDLINE | ID: mdl-38570875

ABSTRACT

BACKGROUND: NODAL signaling plays a critical role in embryonic patterning and heart development in vertebrates. Genetic variants resulting in perturbations of the TGF-ß/NODAL signaling pathway have reproducibly been shown to cause laterality defects in humans. To further explore this association and improve genetic diagnosis, the study aims to identify and characterize a broader range of NODAL variants in a large number of individuals with laterality defects. METHODS: We re-analyzed a cohort of 321 proband-only exomes of individuals with clinically diagnosed laterality congenital heart disease (CHD) using family-based, rare variant genomic analyses. To this cohort we added 12 affected subjects with known NODAL variants and CHD from institutional research and clinical cohorts to investigate an allelic series. For those with candidate contributory variants, variant allele confirmation and segregation analysis were studied by Sanger sequencing in available family members. Array comparative genomic hybridization and droplet digital PCR were utilized for copy number variants (CNV) validation and characterization. We performed Human Phenotype Ontology (HPO)-based quantitative phenotypic analyses to dissect allele-specific phenotypic differences. RESULTS: Missense, nonsense, splice site, indels, and/or structural variants of NODAL were identified as potential causes of heterotaxy and other laterality defects in 33 CHD cases. We describe a recurrent complex indel variant for which the nucleic acid secondary structure predictions implicate secondary structure mutagenesis as a possible mechanism for formation. We identified two CNV deletion alleles spanning NODAL in two unrelated CHD cases. Furthermore, 17 CHD individuals were found (16/17 with known Hispanic ancestry) to have the c.778G > A:p.G260R NODAL missense variant which we propose reclassification from variant of uncertain significance (VUS) to likely pathogenic. Quantitative HPO-based analyses of the observed clinical phenotype for all cases with p.G260R variation, including heterozygous, homozygous, and compound heterozygous cases, reveal clustering of individuals with biallelic variation. This finding provides evidence for a genotypic-phenotypic correlation and an allele-specific gene dosage model. CONCLUSION: Our data further support a role for rare deleterious variants in NODAL as a cause for sporadic human laterality defects, expand the repertoire of observed anatomical complexity of potential cardiovascular anomalies, and implicate an allele specific gene dosage model.


Subject(s)
Heart Defects, Congenital , Heterotaxy Syndrome , Transposition of Great Vessels , Animals , Humans , Arteries , Comparative Genomic Hybridization , Heart Defects, Congenital/genetics , Heterotaxy Syndrome/genetics , Phenotype
7.
J Am Heart Assoc ; 13(9): e032174, 2024 May 07.
Article in English | MEDLINE | ID: mdl-38686874

ABSTRACT

BACKGROUND: A risk model has been proposed to provide a patient individualized estimation of risk for major clinical events (heart failure events, ventricular arrhythmia, all-cause mortality) in patients with transposition of the great arteries and atrial switch surgery. We aimed to externally validate the model. METHODS AND RESULTS: A retrospective, multicentric, longitudinal cohort of 417 patients with transposition of the great arteries (median age, 24 years at baseline [interquartile range, 18-30]; 63% men) independent of the model development and internal validation cohort was studied. The performance of the prediction model in predicting risk at 5 years was assessed, and additional predictors of major clinical events were evaluated separately in our cohort. Twenty-five patients (5.9%) met the major clinical events end point within 5 years. Model validation showed good discrimination between high and low 5-year risk patients (Harrell C index of 0.73 [95% CI, 0.65-0.81]) but tended to overestimate this risk (calibration slope of 0.20 [95% CI, 0.03-0.36]). In our population, the strongest independent predictors of major clinical events were a history of heart failure and at least mild impairment of the subpulmonary left ventricle function. CONCLUSIONS: We reported the first external validation of a major clinical events risk model in a large cohort of adults with transposition of the great arteries. The model allows for distinguishing patients at low risk from those at intermediate to high risk. Previous episode of heart failure and subpulmonary left ventricle dysfunction appear to be key markers in the prognosis of patients. Further optimizing risk models are needed to individualize risk predictions in patients with transposition of the great arteries.


Subject(s)
Arterial Switch Operation , Heart Failure , Transposition of Great Vessels , Humans , Male , Female , Transposition of Great Vessels/surgery , Adult , Risk Assessment/methods , Retrospective Studies , Young Adult , Risk Factors , Arterial Switch Operation/adverse effects , Adolescent , Heart Failure/diagnosis , Reproducibility of Results , Predictive Value of Tests , Arrhythmias, Cardiac/diagnosis , Arrhythmias, Cardiac/physiopathology , Longitudinal Studies , Time Factors
9.
Int J Cardiovasc Imaging ; 40(5): 1157-1158, 2024 May.
Article in English | MEDLINE | ID: mdl-38652393

ABSTRACT

Transposition of the great arteries (TGA) is a cyanotic congenital heart disease characterized by ventriculoarterial discordance and atrioventricular concordance with the great arteries in a parallel relationship. Prenatal diagnosis of TGA has implications for postnatal outcomes, allowing for planned delivery and perinatal management. Three-dimensional virtual or physical models of fetal TGA allow better understanding of fetal cardiac anomalies by parents and interactive discussion among the multidisciplinary team (obstetricians, pediatricians, maternal-fetal specialists, pediatric cardiologists, and cardiovascular surgeons), as well as continuing medical education.


Subject(s)
Fetal Heart , Predictive Value of Tests , Transposition of Great Vessels , Ultrasonography, Prenatal , Transposition of Great Vessels/diagnostic imaging , Transposition of Great Vessels/surgery , Transposition of Great Vessels/physiopathology , Humans , Pregnancy , Fetal Heart/diagnostic imaging , Fetal Heart/physiopathology , Female , Imaging, Three-Dimensional , Models, Cardiovascular , Gestational Age , Patient-Specific Modeling , Prognosis , Image Interpretation, Computer-Assisted
10.
Int J Cardiol ; 407: 132027, 2024 Jul 15.
Article in English | MEDLINE | ID: mdl-38583591

ABSTRACT

BACKGROUND: In patients with transposition of the great arteries and an arterial switch operation (TGA-ASO) right ventricular outflow tract (RVOT) obstruction is a common complication requiring one or more RVOT interventions. OBJECTIVES: We aimed to assess cardiopulmonary exercise capacity and right ventricular function in patients stratified for type of RVOT intervention. METHODS: TGA-ASO patients (≥16 years) were stratified by type of RVOT intervention. The following outcome parameters were included: predicted (%) peak oxygen uptake (peak VO2), tricuspid annular plane systolic excursion (TAPSE), tricuspid Lateral Annular Systolic Velocity (TV S'), right ventricle (RV)-arterial coupling (defined as TAPSE/RV systolic pressure ratio), and N-terminal proBNP (NT-proBNP). RESULTS: 447 TGA patients with a mean age of 25.0 (interquartile range (IQR) 21-29) years were included. Patients without previous RVOT intervention (n = 338, 76%) had a significantly higher predicted peak VO2 (78.0 ± 17.4%) compared to patients with single approach catheter-based RVOT intervention (73.7 ± 12.7%), single approach surgical RVOT intervention (73.8 ± 28.1%), and patients with multiple approach RVOT intervention (66.2 ± 14.0%, p = 0.021). RV-arterial coupling was found to be significantly lower in patients with prior catheter-based and/or surgical RVOT intervention compared to patients without any RVOT intervention (p = 0.029). CONCLUSIONS: TGA patients after a successful arterial switch repair have a decreased exercise capacity. A considerable amount of TGA patients with either catheter or surgical RVOT intervention perform significantly worse compared to patients without RVOT interventions.


Subject(s)
Transposition of Great Vessels , Humans , Male , Female , Transposition of Great Vessels/surgery , Transposition of Great Vessels/physiopathology , Adult , Young Adult , Europe/epidemiology , Ventricular Outflow Obstruction/surgery , Ventricular Outflow Obstruction/physiopathology , Ventricular Outflow Obstruction/diagnostic imaging , Arterial Switch Operation/methods , Arterial Switch Operation/adverse effects , Exercise Tolerance/physiology , Exercise Test/methods , Treatment Outcome , Ventricular Function, Right/physiology , Follow-Up Studies
11.
Article in English | MEDLINE | ID: mdl-38522875

ABSTRACT

En bloc rotation of the outflow tracts or double root translocation offers an anatomic repair of transposition of the great arteries, ventricular septal defect, and left ventricular outflow tract obstruction and closely related forms of double outlet right ventricle. The technical principle is to excise aortic and pulmonary root en bloc, rotate them as a whole by 180°, and reimplant them. The left ventricular outflow tract is enlarged with the patch closing the ventricular septal defect. In our experience, two thirds of the pulmonary valves could be preserved. Growth of the aortic and pulmonary root could be demonstrated in several studies performed by our group. It is still a complex and technically demanding procedure with long cardiopulmonary bypass periods and cross-clamp times. However, perioperative mortality and complications do not differ significantly from other forms of reconstruction. The reoperation rate is significantly lower. Presently, the best time to perform this operation seems to be after the newborn period within the first year of life.


Subject(s)
Double Outlet Right Ventricle , Heart Septal Defects, Ventricular , Transposition of Great Vessels , Infant, Newborn , Humans , Infant , Transposition of Great Vessels/surgery , Transposition of Great Vessels/complications , Treatment Outcome , Rotation , Double Outlet Right Ventricle/complications , Double Outlet Right Ventricle/surgery , Heart Septal Defects, Ventricular/surgery
12.
J Extra Corpor Technol ; 56(1): 16-19, 2024 Mar.
Article in English | MEDLINE | ID: mdl-38488714

ABSTRACT

Early cardiac surgery in neonates and infants with congenital heart disease has been performed since the middle to late years of the twentieth century. To date, there are very few reports of successful congenital heart surgery using cardiopulmonary bypass (CPB) in premature babies less than 1000 g with serious congenital heart disease. Limited information is available in the literature describing perfusion techniques for this extremely fragile patient population. Miniaturization of the CPB circuit contributes to multiple factors that affect this population significantly. These factors include the reduction of patient-to-circuit ratios, volume of distribution of pharmacological agents, management of pressure gradients within the CPB system, and increased tactile control by the attending perfusionist. Careful management of the physiological environment of the patient is of utmost importance and can mitigate risks during CPB, including volume shifts into the interstitial space, electrolyte, and acid-base imbalance, and intracranial hemorrhage. We report perfusion techniques successfully utilized during the surgical repair of transposition of the great arteries for an 800 g, 28-week-old neonate. CPB techniques for the smallest and youngest patients may be executed safely when proper physical, chemical, and perfusion process adjustments are made and managed meticulously.


Subject(s)
Arterial Switch Operation , Cardiac Surgical Procedures , Heart Defects, Congenital , Transposition of Great Vessels , Infant, Newborn , Infant , Humans , Transposition of Great Vessels/surgery , Heart Defects, Congenital/surgery , Cardiac Surgical Procedures/methods , Cardiopulmonary Bypass , Perfusion
13.
Pediatr Cardiol ; 45(5): 1089-1099, 2024 Jun.
Article in English | MEDLINE | ID: mdl-38512489

ABSTRACT

Congenitally corrected transposition of the great arteries (ccTGA) is a rare malformation with diverse morphology. We assessed features of fetuses with ccTGA and evaluated neonatal and pediatric outcomes. This was a retrospective review of fetuses with ccTGA at Birmingham Women's and Children's Hospital born from 2005 to 2019. Of thirty-six fetuses identified, six had unavailable prenatal data, one was postnatally diagnosed with isomerism and 29 fetuses were evaluated. ccTGA without associated cardiac lesions was found in 28% (8/29), ccTGA with significant VSD in 31% (9/29), ccTGA with pulmonary obstruction in 24% (7/29) and ccTGA with complex anomalies in 17% (5/29). Tricuspid regurgitation (TR) was observed in 17% (5/29) and heart block (HB) in 10% (3/29) prenatally. Six, that is 21% underwent genetic testing of which one was abnormal. Five extra-cardiac anomalies were reported prenatally and postnatally. Pregnancy was discontinued in five, of which two had moderate TR. There were thirty-one liveborn. Coarctation of the aorta was found in five postnatally but not suspected prenatally. In one, pulmonary stenosis was underestimated; otherwise, prenatal morphology was confirmed. Cardiac interventions were performed in 77% (24/31) liveborn with 39% (12/31) undergoing neonatal intervention. Overall, 6/31 liveborn died including all three with prenatal heart block and one with TR. Estimated survival for all liveborn at 1, 5 and 10 years was 87% (95% CI 76-100%), 83% (95% CI 72-98%) and 80% (95% CI 66-96%) respectively. Accurate prenatal diagnosis of ccTGA is critical for counseling. Early outcomes are favorable with 77% of liveborn undergoing surgery. Fetuses with prenatal diagnosis of complex associated abnormalities, HB and TR appear to do less well.


Subject(s)
Congenitally Corrected Transposition of the Great Arteries , Ultrasonography, Prenatal , Humans , Female , Retrospective Studies , Pregnancy , Infant, Newborn , Transposition of Great Vessels/diagnosis , Transposition of Great Vessels/diagnostic imaging , Echocardiography , Prenatal Diagnosis/methods , Male
15.
Int J Cardiol ; 405: 131969, 2024 Jun 15.
Article in English | MEDLINE | ID: mdl-38490269

ABSTRACT

BACKGROUND: Functional abnormalities of the ascending aorta (AA) have been mainly reported in young patients who underwent arterial switch operation (ASO) for transposition of the great arteries (TGA). OBJECTIVES: To compare systolic, diastolic brachial and central blood pressures (bSBP, bDBP, cSBP, cDBP), aortic biomechanical parameters, and left ventricular (LV) afterload criteria in adult ASO patients with healthy controls and to assess their relationships with LV remodeling and aortic size. MATERIALS AND METHODS: Forty-one prospectively enrolled patients (16.8 to 35.8 years) and 41 age- and sex-matched healthy volunteers underwent cardiac MRI to assess LV remodeling with simultaneous brachial BP estimation. After MRI, carotid-femoral tonometry was performed to measure pulse wave velocity (cfPWV), cSBP and cDBP for further calculation of pulse pressure (cPP), AA distensibility (AAD), and AA and LV elastance (AAE, LVE). RESULTS: bSBP, bDBP, cSBP,cDBP and cPP were all significantly higher in ASO group than in controls: cSBP (116.5 ± 13.8 vs 106.1 ± 12.0, p < 0.001), cDBP (72.5 ± 6.9 vs 67.1 ± 9.4, p = 0.002), cPP (44.0 ± 12.1 vs 39.1 ± 8.9, p = 0.003) and not related to aortic size. AAD were decreased in ASO patients vs controls (4.70 ± 2.72 vs 6.69 ± 2.16, p < 0.001). LV mass was correlated with bSBP, cSBP, cPP (ρ = 0.48; p < 0.001), while concentric LV remodeling was correlated with AAE (ρ = 0.60, p < 0.001) and LVE (ρ = 0.32, p = 0.04), but not with distensibility. CONCLUSION: Even without reaching arterial hypertension, aortic sBP and PP are increased in the adult TGA population after ASO, altering the pulsatile components of afterload and contributing to LV concentric remodeling.


Subject(s)
Arterial Switch Operation , Transposition of Great Vessels , Ventricular Remodeling , Humans , Transposition of Great Vessels/surgery , Transposition of Great Vessels/physiopathology , Transposition of Great Vessels/diagnostic imaging , Male , Female , Adult , Ventricular Remodeling/physiology , Young Adult , Prospective Studies , Adolescent , Arterial Pressure/physiology , Aorta/diagnostic imaging , Aorta/physiopathology , Pulse Wave Analysis , Magnetic Resonance Imaging, Cine/methods , Pulsatile Flow/physiology
16.
J Am Heart Assoc ; 13(6): e031184, 2024 Mar 19.
Article in English | MEDLINE | ID: mdl-38497437

ABSTRACT

BACKGROUND: Distances between delivery and cardiac services can make the care of fetuses with cardiac disease at risk of acute cardiorespiratory instability at birth a challenge. In 2013 we implemented a fetal echocardiography-based algorithm targeting fetuses considered high risk for acute cardiorespiratory instability at ≤2 hours of birth for delivery in our pediatric cardiac operating room of our children's hospital, and, herein, examine our experience. METHODS AND RESULTS: We reviewed maternal and postnatal medical records of all fetuses with cardiac disease encountered January 2013 to March 2022 considered high risk for acute cardiorespiratory instability. Secondary analysis was performed including all fetuses with diagnoses of d-transposition of the great arteries/intact ventricular septum (d-TGA/IVS) and hypoplastic left heart syndrome (HLHS) encountered over the study period. Forty fetuses were considered high risk for acute cardiorespiratory instability: 15 with d-TGA/IVS and 7 with HLHS with restrictive atrial septum, 4 with absent pulmonary valve syndrome, 3 with obstructed anomalous pulmonary veins, 2 with severe Ebstein anomaly, 2 with thoracic/intracardiac tumors, and 7 others. Pediatric cardiac operating room delivery occurred for 33 but not for 7 (5 with d-TGA/IVS, 2 with HLHS with restrictive atrial septum). For high-risk cases, fetal echocardiography had a positive predictive value of 50% for intervention/extracorporeal membrane oxygenation/death at ≤2 hours and 70% at ≤24 hours. Of "low-risk" cases, 6/46 with d-TGA/IVS and 0/45 with HLHS required intervention at ≤2 hours. Fetal echocardiography for predicting intervention/extracorporeal membrane oxygenation/death at ≤2 hours had a sensitivity of 67%, specificity 93%, and positive and negative predictive values of 80% and 87%, respectively, for d-TGA/IVS, and 100%, 95%, 71%, and 100% for HLHS, respectively. CONCLUSIONS: Fetal echocardiography can predict the need for urgent intervention in a majority with d-TGA/IVS and HLHS and in half of the entire spectrum of high-risk cardiac disease.


Subject(s)
Heart Defects, Congenital , Hypoplastic Left Heart Syndrome , Transposition of Great Vessels , Pregnancy , Infant, Newborn , Female , Humans , Child , Operating Rooms , Fetal Heart/diagnostic imaging , Fetal Heart/surgery , Ultrasonography, Prenatal/methods , Heart Defects, Congenital/diagnostic imaging , Heart Defects, Congenital/surgery , Hypoplastic Left Heart Syndrome/diagnostic imaging , Hypoplastic Left Heart Syndrome/surgery , Retrospective Studies
17.
Echocardiography ; 41(3): e15790, 2024 Mar.
Article in English | MEDLINE | ID: mdl-38456327

ABSTRACT

PURPOSE: Our aim in this study was to investigate the prenatal and postnatal prognosis of double outlet right ventricle (DORV) cases diagnosed prenatally by analyzing the outcomes based on the subtype. METHODS: This study is a retrospective chart review. Cases diagnosed with fetal DORV by prenatal ultrasound in the maternal-fetal medicine department of our hospital between 2014 and 2022 were included. Data on maternal characteristics, fetal echocardiographic features (type of DORV), pregnancy and neonatal outcomes (termination of pregnancy [TOP], intrauterine fetal death [IUD], neonatal death [NND], death in infancy (IND), survival) were collected and analyzed. RESULTS: Ninety-nine cases of prenatally diagnosed cases of DORV were included. The prenatal diagnosis was right in 97% of the liveborn fetuses. The cases were classified into subtypes, including transposition of great arteries (TGA), Fallot, ventricular septal defect (VSD), remote, and heterotaxy types. The cohort consisted of 32.3% TGA type, 19.1% fallot type, 11.1% VSD type, 2% remote type, and 35.3% heterotaxy type of DORV. An additional cardiac anomaly was observed in 87% and an extra-cardiac anomaly was observed in 54% of the cases. When we excluded the cases with heterotaxy type but without any chromosomal abnormality, additional genetic abnormalities were detected in 42% of the remaining cases. Outcome of pregnancy was livebirth in 68/99 (68.7%), IUFD in 5/99 (5.1%), and TOP in 26/99 (26.3%). Postnatal cardiac surgical repair was performed in 48 cases. Survival among livebirths was 39/68 (57.3%). Twenty-nine neonates or infants who had additional cardiac anomalies and/or genetic abnormalities died before any surgical intervention. The postoperative survival rate was 39/48 (81.2%). CONCLUSION: The prognosis in DORV depends on the anatomical subtype, the presence, and severity of associated anomalies. Survival increases in isolated cases without any additional structural or genetic anomalies.


Subject(s)
Double Outlet Right Ventricle , Heart Defects, Congenital , Heart Septal Defects, Ventricular , Transposition of Great Vessels , Pregnancy , Infant , Infant, Newborn , Female , Humans , Double Outlet Right Ventricle/diagnostic imaging , Double Outlet Right Ventricle/surgery , Retrospective Studies , Heart Defects, Congenital/diagnostic imaging , Prognosis
18.
J Cardiovasc Electrophysiol ; 35(4): 694-700, 2024 Apr.
Article in English | MEDLINE | ID: mdl-38332493

ABSTRACT

INTRODUCTION: Management of transvenous leads in patients with congenital heart disease (CHD) can be complicated by venous obstructions and residual shunts. We present our experience performing concurrent lead extraction and dilation/stenting of venous pathways, including patients with complete venous obstruction. METHODS: All cases of concurrent lead extraction and recanalization of vena cavae/baffles between 2017 and 2021 at Boston Children's Hospital were retrospectively included and reviewed for safety and efficacy. RESULTS: Eight patients, 4 female, median 38.5 years of age (range 16.7-49 years) and 81.6 kg weight (range 41.3-97.8 kg) at time of procedure were included. All patients had CHD, a majority (n = 7) having transposition of the great arteries palliated via atrial switch. All leads were removed in their entirety, with most patients having two leads extracted (n = 7). Median lead dwell time was 13.8 years (range 3.6-35.3 years). Three patients had complete obstructions, three required stenting of their innominate veins and three required recanalization of their femoral vessels. Median procedure time was 9.8 h (range 5.4-12.8 h). Complications included blood transfusion (n = 2), arrhythmia (n = 3), pleural effusion (n = 1), and pressure ulcer (n = 1). There were no cardiac perforations, venous tears, or deaths. CONCLUSION: Lead extraction along with dilation and stenting of venous anomalies, though long in duration, proved effective with minimal complications. This combined procedure can safely and effectively resolve complete obstructions secondary to transvenous leads.


Subject(s)
Heart Defects, Congenital , Transposition of Great Vessels , Vascular Diseases , Vascular Malformations , Child , Humans , Female , Adolescent , Young Adult , Adult , Middle Aged , Dilatation , Retrospective Studies , Device Removal/adverse effects , Device Removal/methods , Treatment Outcome
19.
J Cardiothorac Vasc Anesth ; 38(4): 992-1005, 2024 Apr.
Article in English | MEDLINE | ID: mdl-38365467

ABSTRACT

OBJECTIVES: Cardiac surgery on cardiopulmonary bypass (CPB) during the neonatal period can cause perioperative organ injuries. The primary aim of this study was to determine the incidence and risk factors associated with postoperative mechanical ventilation duration and acute lung injury after the arterial switch operation (ASO). The secondary aim was to examine the utility of the Brixia score for characterizing postoperative acute lung injury (ALI). DESIGN: A retrospective study. SETTING: A single-center university hospital. PARTICIPANTS: A total of 93 neonates with transposition of great arteries with intact ventricular septum (dTGA IVS) underwent ASO. INTERVENTIONS: None. MEASUREMENTS AND MAIN RESULTS: From January 2015 to December 2022, 93 neonates with dTGA IVS were included in the study. The cohort had a median age of 4.0 (3.0-5.0) days and a mean weight of 3.3 ± 0.5 kg. About 63% of patients had ≥48 hours of postoperative mechanical ventilation after ASO. Risk factors included prematurity, post-CPB transfusion of salvaged red cells, platelets and cryoprecipitate, and postoperative fluid balance by univariate analysis. The larger transfused platelet volume was associated with the risk of ALI by multivariate analysis. The median baseline Brixia scores were 11.0 (9.0-12.0) and increased significantly in the postoperative day 1 in patients who developed moderate ALI 24 hours after admission to the intensive care unit (15.0 [13.0-16.0] v 12.0 [10.0-14.0], p = 0.046). CONCLUSIONS: Arterial switch operation results in a high incidence of ≥48-hour postoperative mechanical ventilation. Blood component transfusion is a potentially modifiable risk factor. The Brixia scores also may be used to characterize postoperative acute lung injury.


Subject(s)
Acute Lung Injury , Arterial Switch Operation , Transposition of Great Vessels , Infant, Newborn , Humans , Arterial Switch Operation/adverse effects , Retrospective Studies , Respiration, Artificial/adverse effects , Transposition of Great Vessels/surgery , Treatment Outcome
20.
Pediatr Med Chir ; 46(1)2024 Feb 22.
Article in English | MEDLINE | ID: mdl-38389487

ABSTRACT

We present the outcomes of delayed chest closure in neonates who underwent congenital heart surgery under cardiopulmonary bypass. Eighty-one consecutive neonatal patients (age ≤ 28 days) with congenital heart diseases who underwent heart operations and after surgery, chest remained open in the intensive care unit until DCC. Correction of transposition of the great arteries pathology was the most common surgical procedure (48.1% of patients). Median sternal closure time from surgery was 3 (2-4) days. Median age of neonates was 9 (5-12) days. In addition, in 4 cases (4.9%) there was secretion from the surgical site after DCC and after taking cultures, in 2 (2.4%) of the cases a pathogen was identified. Multivariable linear regression analysis (adjusted to gender and CPB) showed that only the age-predicted the sternum closure time (ß=-0.09, 95%CI: - 0.16 to -0.02, p=0.02). In-hospital mortality was 6 (7.4%) patients. Although the DCC in neonates who underwent CHD surgical correction was related to a high mortality rate, only the age of neonates predicted the sternum closure time in the ICU.


Subject(s)
Cardiac Surgical Procedures , Heart Defects, Congenital , Transposition of Great Vessels , Infant, Newborn , Humans , Time Factors , Heart Defects, Congenital/surgery , Cardiac Surgical Procedures/methods , Cardiopulmonary Bypass
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