Contemporary Outcomes of Surgical Repair of Total Anomalous Pulmonary Venous Connection in Patients With Heterotaxy Syndrome

Khan, Muhammad S.; Bryant, Roosevelt; Kim, Sung H.; Hill, Kevin D.; Jacobs, Jeffrey P.; Jacobs, Marshall L.; Pasquali, Sara K.; Morales, David L.S.

doi:10.1016/j.athoracsur.2015.02.035

Cited by 55 publications

(55 citation statements)

References 13 publications

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“…In addition, common atrioventricular valve regurgitation also had a negative impact on cardiac function, and both of these factors deteriorated heart function. To varying degrees, patients with a total anomalous pulmonary venous connection had pulmonary vascular obstructive disease after 6 months; thus, patients undergoing total anomalous pulmonary venous return (TAPVC) repair with a univentricular heart had an operative mortality as high as 48% . The other patient died of Acinetobacter baumannii infection due to the difficulty in extubation postoperatively.…”

Section: Discussionmentioning

confidence: 99%

The efficiency of systemic‐to‐pulmonary shunts in older children with hypoplastic pulmonary arteries

Zhang

Fan

et al. 2019

J Card Surg

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Background: To investigate the effects of systemic-to-pulmonary shunts (SPSs) in older children with hypoplastic pulmonary arteries and the factors affecting the development of the pulmonary arteries.Methods: Eighty-six children (older than 3 years) who received SPSs were retrospectively analyzed. The perioperative parameters, the postoperative diameter of the pulmonary artery were collected, and the factors influencing the growth of the pulmonary arteries after an initial palliative shunt operation were analyzed.Results: Two patients died postoperatively (2.33%), and the pulse oxygen saturation (SpO 2 ) increased from 71.70 ± 6.75% preoperatively to 85.20 ± 11.07% at discharge.During the follow-up period of 56 (10-99) months, 37 patients (43.02%) underwent subsequent procedures, and in the remaining patients, the McGoon ratio was increased from 0.96 ± 0.48 at the surgery to 1.30 ± 0.31 at the final assessment (P < 0.05). Univariate analysis indicated that age younger than 5 years old (P < 0.05), pulmonary artery forward flow (P < 0.05) and a diagnosis of tetralogy of Fallot (P < 0.05) played positive roles in the growth of the pulmonary artery after surgery, while children with a McGoon ratio less than 0.6 showed poor development of the pulmonary arteries (P < 0.05). Multivariate analysis showed that age younger than 5 years old (P < 0.05) and pulmonary artery forward flow (P < 0.05) were positive effectors on the growth of the pulmonary artery. Conclusions: Older children with cyanotic congenital heart disease benefited from a systemic-pulmonary shunt and showed increased postoperative oxygen saturation and development of the pulmonary arteries. Age younger than 5 years and pulmonary artery antegrade flow were the positive factors influencing the growth of the pulmonary arteries postoperatively. K E Y W O R D S children, congenital heart disease, pulmonary hypoplasia, systemic-to-pulmonary shunts (SPSs) 1 | INTRODUCTION Pulmonary artery development is one of the determinants of the surgical indications for many complex congenital heart diseases and an intractable problem in clinical treatment. Systemic-to-pulmonary shunts (SPSs) can increase pulmonary blood flow and improve the growth of the pulmonary artery and have been applied in clinical work for more than seventy years. Recently, with the advancement of surgery, anesthesia, and intensive care, SPSs have been rarely used in patients with biventricular repair, but SPSs have been more commonly

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Section: Discussionmentioning

confidence: 99%

The efficiency of systemic‐to‐pulmonary shunts in older children with hypoplastic pulmonary arteries

Zhang

Fan

et al. 2019

J Card Surg

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“…At our institution, the PG repair is only performed in specific conditions, which creates a selection bias and makes extrapolation of our results challenging. However, PG is usually performed in the cohort that could be considered higher risk (eg, heterotaxy, venous anomalies, genetic syndromes) . Results may, therefore, be better for lower risk cohorts.…”

Section: Discussionmentioning

confidence: 99%

Pulsatile Glenn as long-term palliation for single ventricle physiology patients

Chacón-Portillo

Zhu

Dickerson

et al. 2018

Congenital Heart Disease

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Objective There are limited studies analyzing pulsatile Glenn as a long‐term palliation strategy for single ventricle patients. This study sought to determine their outcomes at a single institution. Design A retrospective review was performed. Setting Study performed at a single pediatric hospital. Patients All single ventricle patients who underwent pulsatile Glenn from 1995 to 2016 were included. Outcome measures Pulsatile Glenn failure was defined as takedown, transplant, or death. Further palliation was defined as Fontan, 1.5, or biventricular repair. Risk factors were assessed by Cox multivariable competing risk analyses. Results Seventy‐eight patients underwent pulsatile Glenn at age 9 months (interquartile range, 5‐14). In total, 28% had heterotaxy, 18% had a genetic syndrome, and 24% had an abnormal inferior vena cava. There were 3 (4%) perioperative mortalities. Further palliation was performed in 41 (53%) patients with a median time‐to‐palliation of 4 years (interquartile range, 3‐5). Pulsatile Glenn failure occurred in 10 (13%) patients with 8 total mortalities. Five‐ and 10‐year transplant‐free survival were 91% and 84%, respectively. At a median follow‐up of 6 years (interquartile range, 2‐8), 27 patients (35%) remained with PG (age 7 years [interquartile range, 3‐11], oxygen saturation 83% ± 4%). Preoperative moderate‐severe atrioventricular valve regurgitation (AVVR) (hazard ratio 7.77; 95% confidence interval 1.80‐33.43; P =.005) and higher pulmonary vascular resistance (hazard ratio 2.59; 95% confidence interval 1.08‐6.15; P =.031) were predictors of pulsatile Glenn failure after adjusting for covariates. Reaching further palliation was less likely in patients with preoperative moderate‐severe AVVR (hazard ratio 0.22, 95% confidence interval 0.08‐0.59; P =.002). Conclusion Pulsatile Glenn can be an effective tool to be used in challenging circumstances, these patients can have a favorable long‐term prognosis without reducing their suitability for further palliation.

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“…8,9 Complex forms of TAPVC, encountered in 4 patients, were associated with heterotaxy syndrome and single ventricle physiology, in concordance with studies concerning surgical approach and postoperative course. 14,15 The diagnosis was established at a mean age of 125 days, later than in similar studies. 2,3,10,16…”

Section: Anatomical Subtypesmentioning

confidence: 92%

Total Anomalous Pulmonary Venous Connection in Children

Făgărășan

Muntean

Gozar

et al. 2016

Journal of Interdisciplinary Medicine

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Introduction:The aim of this study was to study the anatomical types of total anomalous pulmonary venous connection (TAPVC), the associated cardiac and extracardiac congenital malformations, clinical manifestations, and postoperative evolution. Materials and methods: Twenty-four patients with a mean age of 125 days, admitted to the Clinic of Pediatric Cardiology III between January 1, 2009 and December 31, 2015 and diagnosed with TAPVC were included in the study. The patients were evaluated clinically, electrocardiographically and echocardiographically, both pre-and postoperatively. Postoperative evolution was monitored at 1, 3, 6, 12 and 24 months. Results: The anatomical types of TAPVC were: supracardiac in 50% of cases (12 patients), cardiac in 37.5% (9 patients) and mixed type in 12.5% of cases (3 patients). The first clinical manifestation was cyanosis in 72.2% of cases. Surgical correction was performed at a mean age of 37 days in obstructive forms, and 254 days in non-obstructive forms. From the study lot, 8.4% of patients had associated extracardiac malformations (anorectal agenesis and Ivemark syndrome). Early postoperative complications included pulmonary hypertension crisis (60% of cases), supraventricular arrhythmias (35% of cases) and chylothorax (8.4% of cases). Late postoperative complications included: reintervention in 8% of patients with mixed type TAPVC. Conclusions: The most frequently encountered type was supracardiac TAPVC, which had a favorable postoperative evolution. Mixed type TAPVC had the highest rate of reintervention.

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Contemporary Outcomes of Surgical Repair of Total Anomalous Pulmonary Venous Connection in Patients With Heterotaxy Syndrome

Cited by 55 publications

References 13 publications

The efficiency of systemic‐to‐pulmonary shunts in older children with hypoplastic pulmonary arteries

The efficiency of systemic‐to‐pulmonary shunts in older children with hypoplastic pulmonary arteries

Pulsatile Glenn as long-term palliation for single ventricle physiology patients

Total Anomalous Pulmonary Venous Connection in Children

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