Hypoplastic Left Heart Syndrome

2018 ◽  
Author(s):  
M. Navaratnam ◽  
C. Ramamoorthy
Keyword(s):  
Blood Flow ◽  
Hypoplastic Left Heart Syndrome ◽  
Treatment Options ◽  
Surgical Techniques ◽  
The United States ◽  
Left Heart ◽  
Hypoplastic Left Heart ◽  
Factors Affecting ◽  
Unit Management ◽  
Left Heart Syndrome

Approximately 960 babies are born per year in the United States with hypoplastic left heart syndrome. Over the last 20 years, advances in surgical techniques, perioperative care, cardiopulmonary bypass, and intensive care unit management have converted this previously fatal condition to one with a neonatal survival rate of 90% to 92% for standard risk patients. Understanding the factors affecting the balance of pulmonary blood flow and systemic blood flow and ensuring adequate cardiac output and end-organ perfusion is critical to successful outcomes. Extracorporeal membrane oxygenation remains an important support modality following stage I palliation. This chapter discusses this syndrome and describes treatment options.

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.

scholarly journals Myocardial blood flow and viability in children post palliation of hypoplastic left heart syndrome assessed with MRI

2015 ◽  
Vol 17 (S1) ◽  
Author(s):  
Philip Wegner ◽  
Michael Jerosch-Herold ◽  
Christopher Hart ◽  
Eileen Pardun ◽  
Hans-Heiner Kramer ◽  
...  
Keyword(s):  
Blood Flow ◽  
Myocardial Blood Flow ◽  
Hypoplastic Left Heart Syndrome ◽  
Left Heart ◽  
Hypoplastic Left Heart ◽  
Left Heart Syndrome

scholarly journals New Aspects in the Diagnosis and Therapy of Fetal Hypoplastic Left Heart Syndrome

2019 ◽  
Vol 79 (08) ◽  
pp. 863-872 ◽  
Author(s):  
Oliver Graupner ◽  
Christian Enzensberger ◽  
Roland Axt-Fliedner
Keyword(s):  
Hypoplastic Left Heart Syndrome ◽  
Treatment Options ◽  
Left Heart ◽  
Hypoplastic Left Heart ◽  
Non Invasive ◽  
New Findings ◽  
Parental Counseling ◽  
The Right ◽  
Systemic Ventricle ◽  
Left Heart Syndrome

AbstractFetal hypoplastic left heart syndrome (HLHS) is a severe congenital heart disease with a lethal prognosis without postnatal therapeutic intervention or surgery. The aim of this article is to give a brief overview of new findings in the field of prenatal diagnosis and the therapy of HLHS. As cardiac output in HLHS children depends on the right ventricle (RV), prenatal assessment of fetal RV function is of interest to predict poor functional RV status before the RV becomes the systemic ventricle. Prenatal cardiac interventions such as fetal aortic valvuloplasty and non-invasive procedures such as maternal hyperoxygenation seem to be promising treatment options but will need to be evaluated with regard to long-term outcomes. Novel approaches such as stem cell therapy or neuroprotection provide important clues about the complexity of the disease. New aspects in diagnostics and therapy of HLHS show the potential of a targeted prenatal treatment planning. This could be used to optimize parental counseling as well as pre- and postnatal management of affected children.

Understanding the Hybrid Stage I Approach for Hypoplastic Left Heart Syndrome

2016 ◽  
Vol 36 (5) ◽  
pp. 48-55
Author(s):  
Sharon L. Cheatham ◽  
Grace M. Deyo
Keyword(s):  
Blood Flow ◽  
Pulmonary Artery ◽  
Hypoplastic Left Heart Syndrome ◽  
Main Pulmonary Artery ◽  
Median Sternotomy ◽  
Stage I ◽  
Left Heart ◽  
Hypoplastic Left Heart ◽  
Cavopulmonary Anastomosis ◽  
Left Heart Syndrome

Hybrid stage I palliation combines cardiothoracic surgery and interventional transcatheter procedures for treatment of hypoplastic left heart syndrome. The approach is an alternative to the Norwood procedure, the traditional first stage of surgical palliation. Hybrid stage I palliation involves placing bilateral branch pulmonary artery bands and a patent ductus arteriosus stent through a median sternotomy, performed without cardiopulmonary bypass. The purpose of the bands is to control blood flow to the lungs and protect the pulmonary bed while the stent sustains systemic cardiac output. A balloon atrial septostomy is performed to create an atrial septal defect for unobstructed blood flow from the left atrium to the right atrium. The second stage of palliative surgery is the comprehensive stage II, which incorporates removal of the stent and pulmonary artery bands, atrial septectomy, anastomosis of the diminutive ascending aorta to the main pulmonary artery, aortic arch augmentation, and bidirectional cavopulmonary anastomosis. The traditional Fontan procedure completes the series of palliation.

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