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 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.

Pathological changes and myocardial remodelling related to the mode of shunting following surgical palliation for hypoplastic left heart syndrome

2008 ◽  
Vol 18 (4) ◽  
pp. 415-422 ◽  
Author(s):  
Massimo A. Padalino ◽  
Chiara Castellani ◽  
Silvia Toffoli ◽  
Mila Della Barbera ◽  
Ornella Milanesi ◽  
...  
Keyword(s):  
Extracellular Matrix ◽  
Right Ventricle ◽  
Hypoplastic Left Heart Syndrome ◽  
Pulmonary Arteries ◽  
Left Heart ◽  
Pulmonary Shunt ◽  
Hypoplastic Left Heart ◽  
Surgical Palliation ◽  
The Right ◽  
Left Heart Syndrome

AbstractBackgroundThe modification of placing the shunt from the right ventricle to the pulmonary arteries, also known as Sano procedure, has allegedly improved results over the short term in surgical palliation of hypoplastic left heart syndrome with the Norwood procedure. With this in mind, we reviewed autopsied specimens from neonates and children who did not survive after either a classic arterio-pulmonary shunt, or the modified procedure with the shunt placed from the right ventricle to the pulmonary arteries, so as to evaluate the pathological substrates of the remodelling of the systemic right ventricle, assessing any differences induced by the 2 techniques.MethodsWe obtained the hearts from 11 patients with neonatal diagnosis of hypoplastic left heart syndrome who died after the first or second stages of the Norwood sequence of operations, comparing them with 6 normal hearts matched for age and weight. Macroscopic, microscopic and morphometric analysis were performed on each specimen, evaluating the diameter of the myocytes, extracellular matrix remodelling in terms of fibrosis and type of collagen, and vascularization in terms of capillary density.ResultsHypertrophy of the myocytes was significantly increased in the hearts from patients having either a classic arterio-pulmonary or the ventriculo-pulmonary modification of the shunt compared to controls (p < 0.05). Myocardial fibrosis was increased in those having a shunt placed from the right ventricle to the pulmonary arteries when compared to the other 2 groups. The ratio of collagen I to collagen III was similar in those undergoing a classic arterio-pulmonary shunt compared to controls (0.94), but was lower in those having a shunt placed from the right ventricle to the pulmonary arteries (0.61), with an increase in collagen type III. The density of capillaries was lower in those who had undergone a classic arterial shunt when compared to the others.ConclusionWe have shown greater remodelling of the ventricular myocardial extracellular matrix in patients having a shunt from the right ventricle to the pulmonary arteries when compared to those having a classic arterio-pulmonary shunt, with this remodelling progressing even after the neonatal period. This may influence a later suboptimal ventricular performance.

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 Results of heart transplantation following failed staged palliation of hypoplastic left heart syndrome and related single ventricle anomalies

2015 ◽  
Vol 48 (5) ◽  
pp. 792-799 ◽  
Author(s):  
Bahaaldin Alsoufi ◽  
Shriprasad Deshpande ◽  
Courtney McCracken ◽  
Brian Kogon ◽  
Robert Vincent ◽  
...  
Keyword(s):  
Heart Transplantation ◽  
Hypoplastic Left Heart Syndrome ◽  
Single Ventricle ◽  
Left Heart ◽  
Hypoplastic Left Heart ◽  
Left Heart Syndrome

Interstage Home Monitoring After Newborn First-Stage Palliation for Hypoplastic Left Heart Syndrome: Family Education Strategies

2017 ◽  
Vol 37 (2) ◽  
pp. 72-88 ◽  
Author(s):  
Jo Ann Nieves ◽  
Karen Uzark ◽  
Nancy A. Rudd ◽  
Jennifer Strawn ◽  
Anne Schmelzer ◽  
...  
Keyword(s):  
Home Care ◽  
Hypoplastic Left Heart Syndrome ◽  
Single Ventricle ◽  
Early Recognition ◽  
Home Monitoring ◽  
Growth Failure ◽  
Left Heart ◽  
Hypoplastic Left Heart ◽  
Growth And Survival ◽  
Left Heart Syndrome

Children born with hypoplastic left heart syndrome are at high risk for serious morbidity, growth failure, and mortality during the interstage period, which is the time from discharge home after first-stage hypoplastic left heart syndrome palliation until the second-stage surgical intervention. The single-ventricle circulatory physiology is complex, fragile, and potentially unstable. Multicenter initiatives have been successfully implemented to improve outcomes and optimize growth and survival during the interstage period. A crucial focus of care is the comprehensive family training in the use of home surveillance monitoring of oxygen saturation, enteral intake, weight, and the early recognition of “red flag” symptoms indicating potential cardiopulmonary or nutritional decompensation. Beginning with admission to the intensive care unit of the newborn with hypoplastic left heart syndrome, nurses provide critical care and education to prepare the family for interstage home care. This article presents detailed nursing guidelines for educating families on the home care of their medically fragile infant with single-ventricle circulation.

scholarly journals A late diagnosis of Pseudohypoaldosteronism type I in an infant with hypoplastic left heart syndrome presenting with failure to thrive

2021 ◽  
pp. 1-3
Author(s):  
Deani H. McVadon ◽  
John M. Costello ◽  
Katherine E. Twombley ◽  
Sinai C. Zyblewski
Keyword(s):  
Hypoplastic Left Heart Syndrome ◽  
Case Reports ◽  
Peripheral Resistance ◽  
Failure To Thrive ◽  
Type I ◽  
Left Heart ◽  
Hypoplastic Left Heart ◽  
Poor Growth ◽  
Left Heart Syndrome ◽  
Pseudohypoaldosteronism Type

Abstract Pseudohypoaldosteronism type I is caused by a peripheral resistance to aldosterone and can present with electrolyte abnormalities, poor growth, or dehydration. Although a rare disease, several case reports have been published regarding Pseudohypoaldosteronism type I in neonates and infants. We report a case of failure to thrive and hyponatremia in an infant with hypoplastic left heart syndrome who was subsequently found to have Pseudohypoaldosteronism type I.

scholarly journals Noninvasive Imaging in Interventional Cardiology: Hypoplastic Left Heart Syndrome

2021 ◽  
Vol 8 ◽  
Author(s):  
Hannah Bellsham-Revell
Keyword(s):  
Hypoplastic Left Heart Syndrome ◽  
Pulmonary Arteries ◽  
Interventional Cardiology ◽  
Systemic Circulation ◽  
Fontan Circulation ◽  
Active Management ◽  
Left Heart ◽  
Hypoplastic Left Heart ◽  
Systemic Right Ventricle ◽  
Left Heart Syndrome

Hypoplastic left heart syndrome (HLHS) is a spectrum of left heart underdevelopment leaving the left side unable to support the systemic circulation. If active management is pursued, then the condition is managed with staged palliation to the Fontan circulation, leaving a systemic right ventricle. Through all surgical stages, and even after completion of Fontan, there are multiple areas that may require intervention, most frequently the branch pulmonary arteries which are essential to a successful Fontan circulation. Echocardiography is the mainstay of assessment, but there is an increasing use of magnetic resonance imaging (MRI) and computed tomography (CT) particularly in relation to extracardiac structures which can be more challenging with echocardiography. Both MRI and CT require set-up, experience and training, and usually sedation or anesthetic in smaller children, but can provide excellent imaging to guide interventions. Cardiac MRI is also able to quantify right ventricular (RV) function which can be challenging on echocardiography. This article describes the modalities available and their use in assessing patients with HLHS prior to catheter interventions.

Abstract 17246: Predictors for Listing for Transplant in Infants with Hypoplastic Left Heart Syndrome : an analysis of the Single Ventricle Reconstruction Trial

Circulation ◽  
2014 ◽  
Vol 130 (suppl_2) ◽  
Author(s):  
Aparna Kulkarni ◽  
Richard Neugebauer ◽  
Jacqueline M Lamour ◽  
Daphne T Hsu
Keyword(s):  
Hypoplastic Left Heart Syndrome ◽  
Median Time ◽  
Single Ventricle ◽  
Significant Risk ◽  
Wait List ◽  
Left Heart ◽  
Hypoplastic Left Heart ◽  
Time To Death ◽  
Valve Insufficiency ◽  
Left Heart Syndrome

Background: Infants with Hypoplastic Left Heart Syndrome (HLHS) following the Norwood procedure have the worst survival among infant heart transplant (HT) recipients. The Single Ventricle Reconstruction (SVR) trial collected vital status and HT data in 920 eligible patients (pts) and prospective preoperative, operative and post-operative data in 554 randomized pts. This trial provides a unique opportunity to evaluate predictors of the need for HT in HLHS infants. Methods: The public use SVR database was used to analyze factors associated with listing for HT and mortality on the wait list and in HT pts. Pts listed for HT were compared with those who survived without listing. Results: Among 920 eligible pts, 41 were listed for HT and follow up data was available in 33/554 pts. 25 pts underwent HT. The median age at listing was 135 days (6 [[Unable to Display Character: &#8211;]] 713 days). All pts listed were status 1A, except for 2 at status 7 and 1 at status 2. Mean RV fractional area change at birth was significantly lower in the listed group (29±8% vs.35±9, p<0.05). ECMO/ CPR, and need for pacemaker were also more common in listed pts compared to the unlisted group. Gestational age, anatomic diagnosis of aortic atresia, AV valve insufficiency and the number of catheterization interventions were not risk factors for HT listing. The median time from listing to HT was 47 days (2 - 340 days). ECMO or VAD support was used in 10 pts prior to HT. Wait list mortality was 29% with a median time to death of 134 days (10 [[Unable to Display Character: &#8211;]] 707 days). Causes of death on the wait list included cardiac (8 pts), renal (1 pt), surgical (1 pt) and other (2pts). Mortality after HT was 36% with a median time to death of 197 days (48-658 days). Overall mortality after listing, including after HT, was 51%; younger age at listing was associated with increased mortality (292 vs 149 days, p<0.04). Conclusion: HT as a rescue procedure for HLHS in the first year of life carries a significant risk of mortality.

Sign in / Sign up

Export Citation Format

Share Document