Associations Between Day of Admission and Day of Surgery on Outcome and Resource Utilization in Infants With Hypoplastic Left Heart Syndrome Who Underwent Stage I Palliation (from the Single Ventricle Reconstruction Trial)

Background Thrombosis is common in infants undergoing staged surgeries for single‐ventricle congenital heart disease. The reported incidence and timing of thrombosis varies widely, making it difficult to understand the burden of thrombosis and develop approaches for prevention. We aimed to determine the timing and cumulative incidence of thrombosis following the stage I Norwood procedure and identify clinical characteristics associated with thrombosis. Methods and Results We analyzed data from the Pediatric Heart Network Single Ventricle Reconstruction trial from 2005 to 2009 and identified infants with first‐time thrombotic events. In 549 infants, the cumulative incidence of thrombosis was 21.2% (n=57) from stage I through stage II. Most events occurred during stage I (n=35/57, 65%), with a median time to thrombosis of 15 days. We used a Cox proportional hazards model to estimate the association of clinical variables with thrombosis. After adjusting for baseline variables, boys had a higher hazard of thrombosis (adjusted hazard ratio [HR], 2.69; 95% CI, 1.44–5.05; P =0.002), non–hypoplastic left heart syndrome cardiac anatomy was associated with a higher early hazard of thrombosis (adjusted HR, 3.93; 95% CI, 1.89–8.17; P <0.001), and longer cardiopulmonary bypass time was also associated with thrombosis (per 10‐minute increase, adjusted HR, 1.07; 95% CI, 1.01–1.12; P =0.02). Lower oxygen saturation after the Norwood procedure increased the hazard for thrombosis in the unadjusted model (HR, 1.08; 95% CI, 1.02–1.14; P =0.011). Conclusions Thrombosis affects 1 in 5 infants through Stage II discharge, with most events occurring during stage I. Male sex, non–hypoplastic left heart syndrome anatomy, longer cardiopulmonary bypass time, and lower stage I oxygen saturation were associated with thrombosis.

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Faculty Opinions recommendation of Early developmental outcome in children with hypoplastic left heart syndrome and related anomalies: the single ventricle reconstruction trial.

Faculty Opinions – Post-Publication Peer Review of the Biomedical Literature ◽

10.3410/f.716748302.792103353 ◽

2012 ◽

Author(s):

Shakeel Qureshi ◽

Nicholas Hayes

Keyword(s):

Hypoplastic Left Heart Syndrome ◽

Single Ventricle ◽

Developmental Outcome ◽

Left Heart ◽

Hypoplastic Left Heart ◽

Left Heart Syndrome ◽

Early Developmental

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Coil-occlusion of the left ventricle as emergency treatment in failing stage I palliation for hypoplastic left heart syndrome with sinusoids

Catheterization and Cardiovascular Interventions ◽

10.1002/ccd.24354 ◽

2012 ◽

Vol 81 (1) ◽

pp. 119-124 ◽

Cited By ~ 5

Author(s):

Johanna Hummel ◽

Brigitte Stiller ◽

Jochen Grohmann

Keyword(s):

Left Ventricle ◽

Hypoplastic Left Heart Syndrome ◽

Emergency Treatment ◽

Stage I ◽

Left Heart ◽

Hypoplastic Left Heart ◽

Coil Occlusion ◽

Left Heart Syndrome

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Seasonality of Hypoplastic Left Heart Syndrome and Single Ventricle Heart in Poland in the Context of Air Pollution

Journal of Clinical Medicine ◽

10.3390/jcm10153207 ◽

2021 ◽

Vol 10 (15) ◽

pp. 3207

Author(s):

Iwona Strzelecka ◽

Małgorzata Biedrzycka ◽

Filip Franciszek Karuga ◽

Bartosz Szmyd ◽

Katarzyna Batarowicz ◽

...

Keyword(s):

Hypoplastic Left Heart Syndrome ◽

Single Ventricle ◽

Single Case ◽

Winter Season ◽

Left Heart ◽

Target Level ◽

Hypoplastic Left Heart ◽

Factors Affecting ◽

Left Heart Syndrome ◽

Significant Factors

Hypoplastic left heart syndrome (HLHS) and single ventricle (SV) remain a significant cause of cardiac deaths occurring in the first week of life. Their pathogenesis and seasonal frequency are still unknown. Therefore, we attempt to look at the genesis of the HLHS and SV in the context of territorial distribution as well as seasonality. A total of 193 fetuses diagnosed with HLHS and 92 with SV were selected. The frequency was analyzed depending on the year, calendar month, quarter and season (fall-winter vs. spring-summer). The spatial distribution of HLHS and SV in Poland was analyzed. We observed a statistically significant overrepresentation of HLHS formation frequency in March: 27 (14.00%) in comparison to a monthly median of 15 (IQR: 13.75–16.25; p = 0.039), as well as a significantly higher frequency of HLHS in 2007−2009: 65 cases (33.68%) in comparison to the annual mean of 13.79 ± 6.36 (p < 0.001). We noted a higher frequency of SV among parous with the last menstrual period reported in the fall/winter season of 58 vs. 34 in the spring/summer season (p = 0.014). The performed analysis also revealed significant SV overrepresentation in 2008: 11 cases (12.00%) in comparison to the annual mean of 6.57 ± 2.71 (p = 0.016). Every single case of HLHS was observed when the concentration of benzo(a)pyrene and/or PM10 exceeded the acceptable/target level. Our research indicates that both the season and the level of pollution are significant factors affecting the health of parous women and their offspring. The reason why HLHS and SV develop more frequently at certain times of the year remains unclear, therefore research on this topic should be continued, as well as on the effects of PM10 and benzo(a)pyrene exposure.

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Survival After Reconstructive Surgery for Hypoplastic Left Heart Syndrome

Circulation ◽

10.1161/circ.102.suppl_3.iii-136 ◽

2000 ◽

Vol 102 (suppl_3) ◽

Cited By ~ 4

Author(s):

William T. Mahle ◽

Thomas L. Spray ◽

Gil Wernovsky ◽

J. William Gaynor ◽

Bernard J. Clark

Keyword(s):

Hypoplastic Left Heart Syndrome ◽

Reconstructive Surgery ◽

Fontan Procedure ◽

Stage I ◽

Left Heart ◽

Hypoplastic Left Heart ◽

Term Survival ◽

Late Death ◽

Entire Cohort ◽

Left Heart Syndrome

Background —There are limited data regarding the long-term survival of patients who have undergone reconstructive surgery for hypoplastic left heart syndrome (HLHS). We reviewed the 15-year experience at our institution to examine survival in the context of continued improvements in early operative results. Methods and Results —Between 1984 and 1999, 840 patients underwent stage I surgery for HLHS. From review of medical records and direct patient contact, survival status was determined. The 1-, 2-, 5-, 10-, and 15-year survival for the entire cohort was 51%, 43%, 40%, 39%, and 39%, respectively. Late death occurred in 14 of the 291 patients discharged to home after the Fontan procedure, although only 1 patient has died beyond 5 years of age. Heart transplantation after stage I reconstruction was performed in 5 patients. Later era of stage I surgery was associated with significantly improved survival ( P <0.001). Three-year survival for patients undergoing stage I reconstruction from 1995 to 1998 was 66% versus 28% for those patients undergoing surgery from 1984 to 1988. Age >14 days at stage I and weight <2.5 kg at stage I were also associated with higher mortality ( P =0.004 and P =0.01, respectively). Other variables, including anatomic subtype, heterotaxia, and age at subsequent staging procedures, were not associated with survival. Conclusions —Over the 15-year course of this study, early- and intermediate-term survival for patients with HLHS undergoing staged palliation increased significantly. Late death and the need for cardiac transplantation were uncommon.

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Cerebral arterial gas embolism in a patient with hypoplastic left heart syndrome treated with emergent hyperbaric oxygen: case report

Undersea and Hyperbaric Medicine ◽

10.22462/03.20.2020.13 ◽

2020 ◽

pp. 431-434

Author(s):

Mark Binkly ◽

◽

Matthew Kelly ◽

Kevin Hardy ◽

◽

...

Keyword(s):

Hypoplastic Left Heart Syndrome ◽

Hyperbaric Oxygen ◽

Single Ventricle ◽

Case Reports ◽

Pulmonary Arteries ◽

Vena Cava ◽

Left Heart ◽

Hypoplastic Left Heart ◽

Small Areas ◽

Left Heart Syndrome

A 30-year-old female with a history of seizure disorder and hypoplastic left heart syndrome treated with a Norwood procedure in 1986 followed by a modified non-fenestrated Fontan (Left SVC to IVC to pulmonary arteries) with a known baffle leak presented to the emergency department. On day of presentation, the patient became unresponsive, with perioral cyanosis, rightward gaze and a left facial droop near the end of a platelet transfusion. An emergent non-contrast head CT revealed intracranial air in the right MCA distribution. She was taken to the hyperbaric chamber and was treated with a U.S. Navy Table 6 in a multiplace chamber with no extensions. Ten minutes into the treatment patient became more alert and spontaneously asked questions. The following day she was treated with a U.S. Navy Table 5. Patient had repeat CT of the head, which showed resolution of intracerebral gas and small areas of ischemia in right frontal lobe and right caudate. On hospital day five neurologic exam was normal, with 5/5 strength and no residual deficits. Treating the patient was a concern because patient has a single ventricle, in which the pulmonary artery is connected directly to the vena cava. There is very little data regarding the effects of hyperbaric oxygen (HBO2) therapy on single-ventricle physiology. Only two case reports of three pediatric patients treated with HBO2 for CAGE in a similar setting are known. In these cases the patients had improvements in their symptoms following HBO2. These cases and ours indicate HBO2 is feasible and indicated for CAGE in patients with cyanotic congenital heart disease.

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Hybrid Stage I Procedure as Initial Palliation for Neonate With Hypoplastic Left Heart Syndrome and Right Congenital Diaphragmatic Hernia

Seminars in Cardiothoracic and Vascular Anesthesia ◽

10.1177/1089253216687856 ◽

2017 ◽

Vol 21 (2) ◽

pp. 145-151 ◽

Cited By ~ 1

Author(s):

Draginja Cvetkovic ◽

Joseph Giamelli ◽

Michael Lyew ◽

Markus Erb ◽

Suvro Sett ◽

...

Keyword(s):

Congenital Diaphragmatic Hernia ◽

Hypoplastic Left Heart Syndrome ◽

Diaphragmatic Hernia ◽

Stage I ◽

Pulmonary Vasculature ◽

Coronary Perfusion ◽

Left Heart ◽

Hypoplastic Left Heart ◽

Bilateral Pulmonary Artery Banding ◽

Left Heart Syndrome

During the past decade, a hybrid procedure has emerged and dramatically evolved as an alternative stage I palliation to the conventional Norwood procedure in neonates with hypoplastic left heart syndrome (HLHS). The hybrid approach avoids the need for cardiopulmonary bypass (CPB) utilizing stenting of the arterial duct and bilateral pulmonary artery banding. Cerebral and coronary perfusion pressure is maintained, and the pulmonary vasculature is protected from higher systemic pressure. Elimination of risks associated with CPB gains vital time to stabilize the patient and correct coexisting noncardiac anomalies and allows growth in preparation for the later stages of the Fontan pathway. The association of HLHS with right congenital diaphragmatic hernia (CDH) is rare. We report performing a successful hybrid stage I palliation on a neonate with HLHS and severe right CDH.

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