scholarly journals Assessment of pulmonary/systemic blood flow ratio after first-stage palliation for hypoplastic left heart syndrome

2000 ◽  
Vol 120 (1) ◽  
pp. 81-87 ◽  
Author(s):  
Jack Rychik ◽  
David M. Bush ◽  
Thomas L. Spray ◽  
J.William Gaynor ◽  
Gil Wernovsky
Keyword(s):  
Blood Flow ◽  
Hypoplastic Left Heart Syndrome ◽  
Left Heart ◽  
Flow Ratio ◽  
Hypoplastic Left Heart ◽  
Systemic Blood ◽  
Systemic Blood Flow ◽  
Blood Flow Ratio ◽  
Left Heart Syndrome

scholarly journals Interstage management of pulmonary blood flow after the Norwood procedure with right ventricle-to-pulmonary artery conduit

2020 ◽  
Vol 58 (3) ◽  
pp. 551-558
Author(s):  
Takashi Yasukawa ◽  
Takaya Hoashi ◽  
Masataka Kitano ◽  
Masatoshi Shimada ◽  
Kenta Imai ◽  
...  
Keyword(s):  
Blood Flow ◽  
Pulmonary Artery ◽  
Pulmonary Blood Flow ◽  
Norwood Procedure ◽  
Flow Ratio ◽  
Hypoplastic Left Heart ◽  
Systemic Blood ◽  
Systemic Blood Flow ◽  
Blood Flow Ratio ◽  
Left Heart Syndrome

Abstract OBJECTIVES Our goal was to assess the efficacy of managing pulmonary blood flow from the Norwood procedure with a right ventricle-to-pulmonary artery (RV–PA) conduit until stage 2 palliation (S2P). METHODS Among 48 consecutive patients undergoing the Norwood procedure between 2008 and 2018, 40 (83.3%) patients who survived to discharge were included in this study. The primary diagnosis was hypoplastic left heart syndrome in 28 (70%) patients and hypoplastic left heart syndrome variant in 12 (30%) patients. All patients received bilateral pulmonary artery banding. The median age and weight at the time of the Norwood procedure were 41 (25th–75th percentiles: 27–89) days and 3.2 (2.7–3.9) kg, respectively. In keeping with institutional strategy, S2P was undertaken when body weight exceeded 5.0 kg, and normal gross motor development was confirmed. RESULTS The RV–PA conduit was clipped in 28 (70%) patients during the perioperative period of the Norwood procedure, then partial unclipping was performed in 8 (20%) patients and full unclipping was performed in 20 (50%) patients. Before S2P, the median pulmonary-to-systemic blood flow ratio was 1.0 (0.7–1.3). The median age and weight at the time of S2P were 10.7 (9.0–12.9) months and 6.3 (5.5–7.1) kg, respectively. The survival rate 5 years after Norwood discharge was 85.3%. Pre-S2P pulmonary-to-systemic blood flow ratio was linearly correlated with greater interstage changes in systemic atrioventricular valve regurgitation (R2 = 0.223, P = 0.004). CONCLUSIONS Interstage management of pulmonary blood flow by RV–PA conduit clipping and gradual unclipping provided good interstage outcomes. The median pulmonary-to-systemic blood flow ratio could be controlled to 1.0 at pre-S2P catheter examination.

Neonatal hypertrophic cardiomyopathy with duct-independent systemic blood flow

1997 ◽  
Vol 7 (4) ◽  
pp. 446-449
Author(s):  
Kenji Suda ◽  
Darlene P. Horton ◽  
Norman H. Silverman
Keyword(s):  
Blood Flow ◽  
Hypertrophic Cardiomyopathy ◽  
Ventricular Hypertrophy ◽  
Upper Body ◽  
Left Heart ◽  
Cavity Size ◽  
Hypoplastic Left Heart ◽  
Systemic Blood ◽  
Systemic Blood Flow ◽  
Left Heart Syndrome

AbstractWe describe a newborn who had echocardiogaphic features of severe hypertrophic cardiomyopathy and duct-dependent systemic blood flow. Blood flow to the upper body could be maintained only by prostaglandin El, as in the case of hypoplastic left heart syndrome. Forward flow was gradually established from the left ventricle, and the patient weaned from prostaglandin El. Although the patient's mother had poorly controlled diabetes, the patient at 24 months of age shows residual but lessened ventricular hypertrophy and increased cavity size.

Outcomes following prenatal diagnosis of hypoplastic left heart syndrome

2002 ◽  
Vol 13 (3) ◽  
pp. 169-180 ◽  
Author(s):  
David A Somerset ◽  
Katherine J Barber ◽  
Mark D Kilby
Keyword(s):  
Hypoplastic Left Heart Syndrome ◽  
Cardiac Disease ◽  
Neonatal Death ◽  
Ductus Arteriosus ◽  
Left Heart ◽  
Hypoplastic Left Heart ◽  
Systemic Blood ◽  
Systemic Blood Flow ◽  
Congenital Cardiac Disease ◽  
Left Heart Syndrome

Hypoplastic Left Heart Syndrome (HLHS) was first described fifty years ago. It is characterised by underdevelopment of the left ventricle with associated aortic and mitral valve hypoplasia or atresia, and varying degrees of hypoplasia of the aortic arch (Figure 1). In utero a physiological right to left shunt of oxygenated blood through the ductus arteriosus bypasses the obstruction and allows normal fetal growth. Closure of the ductus arteriosus in the post-natal period interrupts systemic blood flow, resulting in rapid deterioration and death. Untreated it is a universally fatal condition, leading to neonatal death within the first few days or weeks of life. Although HLHS affects only one baby in 10,000 and accounts for less than 10% of all congenital heart disease, HLHS is responsible for 25% of all deaths due to congenital cardiac disease occurring within the first week of life.

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

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.

Modeling of the Norwood circulation: effects of shunt size, vascular resistances, and heart rate

2001 ◽  
Vol 280 (5) ◽  
pp. H2076-H2086 ◽  
Author(s):  
Francesco Migliavacca ◽  
Giancarlo Pennati ◽  
Gabriele Dubini ◽  
Roberto Fumero ◽  
Riccardo Pietrabissa ◽  
...  
Keyword(s):  
Heart Rate ◽  
Blood Flow ◽  
Pulmonary Vascular Resistance ◽  
Vascular Resistance ◽  
Compartmental Analysis ◽  
Hypoplastic Left Heart ◽  
Systemic Blood Flow ◽  
Initial Procedure ◽  
Blood Flow Ratio ◽  
Left Heart Syndrome

Hypoplastic left heart syndrome is the most common lethal cardiac malformation of the newborn. Its treatment , apart from heart transplantation , is the Norwood operation. The initial procedure for this staged repair consists of reconstructing a circulation where a single outlet from the heart provides systemic perfusion and an interpositioning shunt contributes blood flow to the lungs. To better understand this unique physiology, a computational model of the Norwood circulation was constructed on the basis of compartmental analysis. Influences of shunt diameter, systemic and pulmonary vascular resistance, and heart rate on the cardiovascular dynamics and oxygenation were studied. Simulations showed that 1) larger shunts diverted an increased proportion of cardiac output to the lungs, away from systemic perfusion, resulting in poorer O2 delivery, 2) systemic vascular resistance exerted more effect on hemodynamics than pulmonary vascular resistance, 3) systemic arterial oxygenation was minimally influenced by heart rate changes, 4) there was a better correlation between venous O2 saturation and O2 delivery than between arterial O2 saturation and O2delivery, and 5) a pulmonary-to-systemic blood flow ratio of 1 resulted in optimal O2 delivery in all physiological states and shunt sizes.

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