scholarly journals Anesthesia Management in VP Shunt Surgery in Neonates with Hypoplastic Left Heart Syndrome (HLHS) and Dandy Walker Syndrome

2021 ◽  
Vol 1 (1) ◽  
pp. 7
Author(s):  
Purwoko Purwoko ◽  
Fandi Ahmad Muttaqin
Keyword(s):  
Patent Ductus Arteriosus ◽  
Hypoplastic Left Heart Syndrome ◽  
Vascular Resistance ◽  
Ductus Arteriosus ◽  
Clinical Manifestations ◽  
Heart Sounds ◽  
Left Heart ◽  
Hypoplastic Left Heart ◽  
Left Heart Syndrome ◽  
Patent Ductus

<p><strong>Introduction:</strong> Hypoplastic left heart syndrome (HLHS) is defined as the incomplete development of the left heart structures, including the mitral valve, left ventricle, aortic valve, and aortic arch. The clinical presentation of HLHS depends on the patency of the ductus arteriosus and the degree of restriction of the atrial septum. Common clinical manifestations include cyanosis, respiratory distress, and hemodynamic instability within hours of delivery. Delays in diagnosis and delay in intervention will increase the morbidity of neonates with HLHS.</p><p><strong>Purpose:</strong> To understand the mechanism of HLHS along with the principles of anesthesia in neuroaesthetics procedures in pediatrics in order to obtain a good outcome.</p><p><strong>Case Illustration:</strong> A 9-day old baby girl, weight 2522 grams with HLHS (aortic atresia) with patent ductus arteriosus (PDA), type II atrial septal defect (ASD) with a left to right shunt, and dandy walker syndrome. From the physical examination, the patient's condition is stable, with GCS E4V5M6, pulse 130-135 beats/minute, breath rate 48x / minute, and SpO2 94-98%. The patient's head was enlarged from birth, no heart sounds were found, regular I-II heart sounds. Blood laboratory tests showed a total bilirubin value of 11.7 mg / dL, direct bilirubin 0.64 mg / dL, indirect bilirubin 10.43 mg / dL, hemoglobin 20.1 g%, hematocrit 55%, with leukocytes 19.9x103 / L, platelets 216 x 103 / L, serum Na 122 mmol / L, K 7.8 mmol / L, Cl 101 mmol / L.</p><p><strong>Discussion:</strong> The anesthetic approach in HLHS is to maintain preoperative hemodynamic stability by maintaining heart rate, preload, and PGE1, balancing systemic vascular resistance and pulmonary vascular resistance, preventing too high PaO2, and administering inotropic agents to increase cardiac output and keep the patent ductus arteriosus open. The balance of systemic and pulmonary blood flow is a key principle in the management of HLHS anesthesia. </p><p><strong>Conclusion:</strong> The main goal of HLHS anesthesia is to minimize hemodynamic changes to prevent compromised hemodynamics in both circulations and maintaining stability is essential in preventing morbidity, complications, and increasing good outcomes in surgery..</p><p> </p><p> </p>

Predictors of Ductus Arteriosus In-Stent Stenosis in the Hybrid Approach to Hypoplastic Left Heart Syndrome

2012 ◽  
Vol 34 (3) ◽  
pp. 656-660 ◽  
Author(s):  
Holly A. Nadorlik ◽  
Matthew J. Egan ◽  
Sharon L. Hill ◽  
John P. Cheatham ◽  
Mark Galantowicz ◽  
...  
Keyword(s):  
Hypoplastic Left Heart Syndrome ◽  
Ductus Arteriosus ◽  
Hybrid Approach ◽  
Left Heart ◽  
Hypoplastic Left Heart ◽  
Left Heart Syndrome ◽  
Stent Stenosis

scholarly journals Proximal ductus arteriosus stenosis after the hybrid stage I procedure in a newborn with hypoplastic left heart syndrome

2013 ◽  
Vol 2 ◽  
pp. 187-189
Author(s):  
Maciej Chojnicki ◽  
Ireneusz Haponiuk ◽  
Mariusz Steffens ◽  
Radoslaw Jaworski ◽  
Aneta Szofer-Sendrowska ◽  
...  
Keyword(s):  
Hypoplastic Left Heart Syndrome ◽  
Ductus Arteriosus ◽  
Stage I ◽  
Left Heart ◽  
Hypoplastic Left Heart ◽  
Left Heart Syndrome

scholarly journals Hypoplastic Left Heart Syndrome: Diagnostic Difficulties in Prenatal Period

2021 ◽  
Vol 18 (5) ◽  
pp. 416-422
Author(s):  
Nodira M. Normuradova ◽  
Аkram A. Fazilov ◽  
Munisa M. Rasulova
Keyword(s):  
Hypoplastic Left Heart Syndrome ◽  
Color Doppler ◽  
Ductus Arteriosus ◽  
Interventricular Septum ◽  
Diagnostic Errors ◽  
Prenatal Period ◽  
Left Heart ◽  
Hypoplastic Left Heart ◽  
Atrioventricular Canal ◽  
Left Heart Syndrome

The article discusses the possible diagnostic errors in the hypoplastic left heart syndrome (HLHS) with mitral atresia and intact interventricular septum. “Atrioventricular canal defects” can be commonly and mistakenly diagnosed the prenatal period in such cases. The aspects and possibilities of differential diagnosis of these defects in the prenatal period are discussed. Ultrasound sign is presented in the article, we have named it “hockey stick with puck” that characterizes the retrograde blood flow in the aortic arch. This sign is diagnosed by color Doppler mapping of the sagittal section of the ductus arteriosus and can serve as a marker of mitral/aortic atresia.

Computational Modeling of Hypoplastic Left Heart Syndrome (HLHS) in Newborn Babies

2011 ◽  
Author(s):  
Ali Jalali ◽  
C. Nataraj ◽  
Gerard F. Jones ◽  
Daniel J. Licht
Keyword(s):  
Cardiac Output ◽  
Pulmonary Artery ◽  
Hypoplastic Left Heart Syndrome ◽  
Ductus Arteriosus ◽  
Compartmental Analysis ◽  
Electrical Circuit ◽  
Lumped Parameter Model ◽  
Left Heart ◽  
Hypoplastic Left Heart ◽  
Left Heart Syndrome

Hypoplastic left heart syndrome (HLHS) is a congenital heart defect (CHD) in which left side of the heart is severely underdeveloped. To better understand this unique physiology, a computational model of the hypoplastic heart was constructed on the basis of compartmental analysis. Lumped parameter model of HLHS is developed based on the electrical circuit analogy. Model is made up of three parts: hypoplastic heart, pulmonary circulation and systemic circulation. Plots of blood pressure and flow for various parts of body show great match between predicted values and what we expected for the case of HLHS babies. Influence of patent ductus arteriosus (PDA) and ASD resistances on cardiac output and pulmonary to systemic flow was also studied. Results show that by increasing the PDA resistance causes more flow to pulmonary compartments and so the ratio increases. Blood flow increases by decreasing of pulmonary artery resistant. Increasing the PDA resistance causes decrease the cardiac output because of more resistance against blood occurs. Saturation increases by decreasing of pulmonary artery resistant.

Comparison of self-expandable and balloon-expanding stents for hybrid ductal stenting in hypoplastic left heart complex

2016 ◽  
Vol 27 (5) ◽  
pp. 837-845 ◽  
Author(s):  
Sebastian Goreczny ◽  
Shakeel A. Qureshi ◽  
Eric Rosenthal ◽  
Thomas Krasemann ◽  
Mohamed S. Nassar ◽  
...  
Keyword(s):  
Hypoplastic Left Heart Syndrome ◽  
Ductus Arteriosus ◽  
Left Heart ◽  
Stent Group ◽  
Hypoplastic Left Heart ◽  
Group 4 ◽  
Left Heart Syndrome ◽  
Term Performance ◽  
Hypoplastic Left Heart Complex

AbstractObjectivesWe aimed to compare the procedural and mid-term performance of a specifically designed self-expanding stent with balloon-expandable stents in patients undergoing hybrid palliation for hypoplastic left heart syndrome and its variants.BackgroundThe lack of specifically designed stents has led to off-label use of coronary, biliary, or peripheral stents in the neonatal ductus arteriosus. Recently, a self-expanding stent, specifically designed for use in hypoplastic left heart syndrome, has become available.MethodsWe carried out a retrospective cohort comparison of 69 neonates who underwent hybrid ductal stenting with balloon-expandable and self-expanding stents from December, 2005 to July, 2014.ResultsIn total, 43 balloon-expandable stents were implanted in 41 neonates and more recently 47 self-expanding stents in 28 neonates. In the balloon-expandable stents group, stent-related complications occurred in nine patients (22%), compared with one patient in the self-expanding stent group (4%). During follow-up, percutaneous re-intervention related to the ductal stent was performed in five patients (17%) in the balloon-expandable stent group and seven patients (28%) in self-expanding stents group.ConclusionsHybrid ductal stenting with self-expanding stents produced favourable results when compared with the results obtained with balloon-expandable stents. Immediate additional interventions and follow-up re-interventions were similar in both groups with complications more common in those with balloon-expandable stents.

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.

Preoperative Venovenous ECMO in an Infant With Late Diagnosis of Hypoplastic Left Heart Syndrome

2017 ◽  
Vol 11 (4) ◽  
pp. NP41-NP43
Author(s):  
Lena K. Eschenbach ◽  
Jelena Kasnar-Samprec ◽  
Kilian Ackermann ◽  
Christian Schreiber ◽  
Rudiger Lange ◽  
...  
Keyword(s):  
Hypoplastic Left Heart Syndrome ◽  
Ductus Arteriosus ◽  
Late Diagnosis ◽  
Left Heart ◽  
Hypoplastic Left Heart ◽  
Venovenous Extracorporeal Membrane Oxygenation ◽  
Venovenous Ecmo ◽  
Stage 1 ◽  
Left Heart Syndrome ◽  
Late Referral

Late diagnosis and late referral for the Norwood stage 1 procedure in patients with hypoplastic left heart syndrome is rare and associated with a higher mortality. We present a case of a cyanotic almost five-week-old infant with hypoplastic left heart syndrome, highly restrictive foramen ovale, and patent ductus arteriosus, who was bridged with venovenous extracorporeal membrane oxygenation to the Norwood stage 1 procedure.

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