Neurodevelopmental outcome of patients after the Fontan operation: A comparison between children with hypoplastic left heart syndrome and other functional single ventricle lesions

2000 ◽  
Vol 137 (5) ◽  
pp. 646-652 ◽  
Author(s):  
Caren S. Goldberg ◽  
Edward M. Schwartz ◽  
James A. Brunberg ◽  
Ralph S. Mosca ◽  
Edward L. Bove ◽  
...  
Keyword(s):  
Hypoplastic Left Heart Syndrome ◽  
Single Ventricle ◽  
Fontan Operation ◽  
Neurodevelopmental Outcome ◽  
Left Heart ◽  
Hypoplastic Left Heart ◽  
Left Heart Syndrome

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

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.

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

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.

scholarly journals Geometry of the pulmonary arteries before the Fontan operation: can we influence it during the Norwood procedure?

2020 ◽  
Vol 57 (6) ◽  
pp. 1098-1104
Author(s):  
Katarzyna Januszewska ◽  
Pawel Nawrocki ◽  
Anja Lehner ◽  
Julia Stegger ◽  
Felix Kleinerueschkamp ◽  
...  
Keyword(s):  
Hypoplastic Left Heart Syndrome ◽  
Fontan Operation ◽  
Pulmonary Arteries ◽  
Norwood Procedure ◽  
Left Heart ◽  
Hypoplastic Left Heart ◽  
Second Stage ◽  
The Right ◽  
Left Heart Syndrome ◽  
The Impact

Abstract OBJECTIVES The right ventricle-to-pulmonary artery (RV-PA) shunt provides stable haemodynamics after the Norwood procedure but can influence development of the central pulmonary arteries (PAs). The goal of this study was to analyse the geometry of the central PAs in children with hypoplastic left heart syndrome before the Fontan operation with respect to the RV-PA shunt site and the type of the second-stage operation. METHODS A total of 161 children with hypoplastic left heart syndrome, median age 2.7 (range 1.3–9.8) years and median weight 12.7 (range 7.6–26.1) kg, underwent the Fontan operation after having had the Norwood procedure with an RV-PA shunt. The patients were divided into 2 groups: left-sided RV-PA (L-RV-PA) (n = 129) with the shunt on the left and right-sided RV-PA (n = 32) with the shunt on the right side of the neoaorta. Angiographic data obtained before the Fontan and all cardiac catheterization interventions were analysed retrospectively. RESULTS Between the second and third stages, as well as directly before the Fontan operation, the L-RV-PA group required more PA catheter interventions (P = 0.001 and P = 0.03). In this group, the minimal left PA diameter was smaller than that in the R-RV-PA group (P = 0.021). Leaving the shunt open until the Fontan operation increased the rate of PA interventions in the L-RV-PA group (P = 0.001), but there is no evidence of the impact on the development of the left PAs (P = 0.075). There is also no evidence that the type of the second-stage procedure influences the intervention rate before the Fontan procedure (P = 0.14). CONCLUSIONS Children who have the L-RV-PA shunt require more PA catheter interventions. The right-sided RV-PA shunt and the subsequent Glenn anastomosis in the place of the shunt are associated with distortion-free and more symmetrical development of the central PAs.

scholarly journals Fontan operation for hypoplastic left heart syndrome with absent aortic valve

2004 ◽  
Vol 128 (2) ◽  
pp. 315-316 ◽  
Author(s):  
Narutoshi Hibino ◽  
Yorikazu Harada ◽  
Takeshi Hiramatsu ◽  
Satoshi Yasukochi ◽  
Gengi Satomi
Keyword(s):  
Aortic Valve ◽  
Hypoplastic Left Heart Syndrome ◽  
Fontan Operation ◽  
Left Heart ◽  
Hypoplastic Left Heart ◽  
Absent Aortic Valve ◽  
Left Heart Syndrome

scholarly journals Tricuspid annular plane systolic excursion correlates with exercise capacity in a cohort of patients with hypoplastic left heart syndrome after Fontan operation

2016 ◽  
Vol 33 (12) ◽  
pp. 1897-1902 ◽  
Author(s):  
David J. Goldberg ◽  
Benjamin French ◽  
Anita L. Szwast ◽  
Michael G. McBride ◽  
Stephen M. Paridon ◽  
...  
Keyword(s):  
Hypoplastic Left Heart Syndrome ◽  
Exercise Capacity ◽  
Fontan Operation ◽  
Left Heart ◽  
Hypoplastic Left Heart ◽  
Left Heart Syndrome
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