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At least 19 recordsLinked to original sources
PubMed2001

Two-Ventricle repair for hypoplastic left heart syndrome.

Hypoplastic left heart syndrome encompasses a spectrum of structural cardiac malformations characterized by severe underdevelopment of the structures in the left heart-aorta complex. The severe end of the spectrum consists of aortic atresia and mitral atresia, whereas at the mild end patients have aortic valve and mitral valve hypoplasia without intrinsic valve stenosis, recently described as hypoplastic left heart complex. These patients may be candidates for biventricular repair. Since November 1988, 12 of 13 patients (92%) with hypoplastic left heart complex have undergone two-ventricle repair and one patient the Norwood operation. Median age at surgery was 7 days (5 to 45 days) and median weight was 3.59 kg (2.6 to 4.3 kg). Biventricular repair consisted of pulmonary homograft patch aortoplasty of the aortic arch and ascending aorta and closure of the interatrial and interventricular communications. The left heart was able to support the systemic circulation in 11 of 12 patients undergoing biventricular repair (92%). The early mortality was two patients (15.4%). The mean follow-up was 67 months (range, 11 months to 133 months). There was one late death at 39 months. Reoperations were necessary in four patients for left ventricular outflow tract obstruction and in two patients for recurrent coarctation. We have successfully achieved biventricular repair in most patients with hypoplastic left heart complex, a subset of patients with hypoplastic left heart syndrome. Reoperation, particularly for left ventricular outflow tract obstruction, appears likely. Finally, with increased experience, predictive criteria for the feasibility and optimal outcome of biventricular repair will be more accurately defined.

Aorta↗
PubMed2000

Hypoplastic left heart syndrome.

Hypoplastic left heart syndrome may be accurately diagnosed during fetal life. Prenatal diagnosis provides the opportunity for parents to make an informed choice about their options, including surgery, nonintervention postnatally or termination of pregnancy. Short to medium term survival continues to improve for a condition that was previously invariably lethal. There continues to be a significant mortality and morbidity associated with hypoplastic left heart syndrome, and the long-term prognosis is unknown. Knowledge of the condition prior to birth means that babies who are to undergo surgery present in optimal condition for such interventions. Parents who have had an affected fetus or child should be offered detailed fetal echocardiography to exclude a recurrence in subsequent pregnancies.

Aortic Coarctation↗
PubMed1982

[Echocardiographic assessment of anatomical detail in patients with hypoplastic left heart syndrome].

Hypoplastic left heart syndrome is a disease with poor prognosis, which is characterized by severe heart failure in early neonatal period. However, there are some patients who survive for relatively longer period. In the light of this fact, 18 patients with this syndrome were studied by echocardiography, and the anatomical and functional classification was attempted. Hypoplastic left heart syndrome was classified into three types: type I was mitral atresia and aortic atresia, type II was mitral atresia and aortic stenosis, and type III was mitral atresia with an abnormality of cono-truncal relationship, respectively. M-mode echocardiography was performed in 15 of the 18 patients, and two-dimensional echocardiograms were recorded in seven cases. Heart catheterization was performed in all patients. Autopsy was performed on 14 of the 18 patients, and its findings were compared with the findings obtained by echocardiography. Two-dimensional echocardiographic classification of this syndrome was possible in all the patients using above-mentioned criteria. Patent ductus arteriosus was detected in two of six cases having this syndrome, and foramen ovale in 4 of 5 cases. The morphological evaluation of both the interatrial and interventricular septum was made by two-dimensional echocardiography, and it contributed to decide the indication of balloon-atrial septostomy. The patients with type I had the poorest prognosis, and the patients with type III survived longer period. Anatomical classification of this syndrome and detection of associated anomalies by two-dimensional echocardiography are recommended to consider the prognostic and surgical considerations.

Adolescent↗
PubMed2005

Prenatal sonographic features of isolated hypoplastic left heart syndrome.

Hypoplastic left heart syndrome (HLHS) is a spectrum of fetal conditions associated with severe hypoplasia of the left ventricle and left ventricular outflow tract. The purpose of this series was to focus on the sonographic prenatal features of isolated HLHS. Based on the 5 cases presented here, the prenatal sonographic features of HLHS include small-sized left ventricle, atretic or hypoplastic mitral valves with restricted motion and a small amount of antegrade flow or regurgitation, and hypoplastic ascending aorta. Retrograde flow in the transverse aortic arch strongly suggests inadequate systemic output from the left heart. Tricuspid regurgitation is common and increases the chance for the development of hydrops. Other findings may be helpful, including left ventricular hyperechoic endocardium and increased nuchal translucency at 11-14 weeks. With careful fetal echocardiography, HLHS may be readily observed on a 4-chamber view as either a small or even absent left ventricle.

Abortion, Eugenic↗
PubMed2003

Spinal anaesthesia in a child with hypoplastic left heart syndrome.

Hypoplastic left heart syndrome (HLHS), a complex congenital heart disease, is the most common lethal cardiac defect in neonates. Its treatment includes cardiac transplantation and/or surgical palliation. Associated extracardiac congenital abnormalities are exceptional. We report the case of a neonate with HLHS and anorectal atresia who required urgent surgical management to relieve intestinal obstruction. The surgery was successfully performed under spinal anaesthesia.

Anesthesia, Spinal↗
PubMed2000

Congenital Heart Surgery Nomenclature and Database Project: hypoplastic left heart syndrome.

Hypoplastic left heart syndrome (HLHS) encompasses a spectrum of structural cardiac malformations that are characterized by severe underdevelopment of the structures in the left heart-aorta complex, including the left ventricular cavity and mass. The severe end of the spectrum consists of aortic atresia and mitral atresia with a nonexistent left ventricle, whereas at the mild end patients have aortic valve and mitral valve hypoplasia without intrinsic valve stenosis, and milder degrees of left ventricular hypoplasia, recently described as hypoplastic left heart complex (HLHC). Although the overwhelming majority of the patients can only have a univentricular repair, a small minority of patients with HLHS, particularly those that are described as having HLHC, may be candidates for biventricular repair. In this paper, the extant nomenclature for HLHS is reviewed for the purpose of establishing a unified reporting system. The subject was debated and reviewed by members of the STS-Congenital Heart Surgery Nomenclature and Database Committee and representatives from the European Association for Cardiothoracic Surgery. Efforts were made to include all relevant nomenclature categories using synonyms where appropriate. A comprehensive database set is presented, which is based on a hierarchical scheme. Data are entered at various levels of complexity and detail that can be determined by the clinician. These data can lay the foundation for comprehensive risk stratification analyses. A minimum database set is also presented which will allow for data sharing, and would lend itself to basic interpretation of trends. Outcome tables relating diagnoses, procedures, and various risk factors are presented.

Databases, Factual↗
PubMed2005

Preoperative management of hypoplastic left heart syndrome.

Hypoplastic left heart syndrome (HLHS) is the most common functional single ventricle congenital cardiac defect. This syndrome is characterised by a functional single right ventricle and systemic outflow obstruction. The systemic and pulmonary circulations compete for cardiac output with a resultant precarious balance among systemic, pulmonary and coronary blood flows. A once fatal diagnosis, advances in operative and perioperative care have resulted in a dramatic improvement in survival. The preoperative management of neonates with HLHS is based predominately on clinical experience and extrapolated data from the postoperative literature. Management focuses on maintaining patency of the systemic outflow, balancing the pulmonary and systemic blood flows, and preserving the function of a single right ventricle to maximise oxygen delivery to the tissues. This paper reviews the available therapies for the preoperative management of HLHS.

Cardiovascular Agents↗
PubMed2002

The influence of a restrictive atrial septal defect on pulmonary vascular morphology in patients with hypoplastic left heart syndrome.

Hypoplastic left heart syndrome (HLHS) with a restrictive atrial septal defect (ASD) is a form of congenital heart disease with considerable morbidity and mortality. This morphologic analysis assesses the pulmonary vasculature in this patient population. Pulmonary arteries, the persistence of high-resistance fetal arterioles, pulmonary veins, and lymphatics from multiple lung sections from each of five patients with HLHS and a restrictive ASD were compared to those of five patients with HLHS and nonrestrictive ASD. Lung sections from each patient were qualitatively graded in severity of pathology from 0 to 3 for each of the structures described previously, with the pathologist blinded to the status of the ASD. Patients with a restrictive ASD exhibited more significant pulmonary venous thickening and lymphatic dilatation (p = 0.02), with a tendency toward persistence of high-resistance fetal vessels (p = 0.2), compared to patients with a nonrestrictive ASD. These findings imply that patients with HLHS and a restrictive ASD possess pulmonary vascular abnormalities that place them at higher risk for the current surgical interventions available compared to patients with a nonrestrictive ASD.

Female↗
PubMed2002

[Ethical, psycho-social, legislative and economic aspects of surgical treatment of the hypoplastic left heart syndrome].

Hypoplastic left heart syndrome (HLHS) represents a lethal congenital heart disease, which used to be considered inoperable. The improvement of results of cardiac surgery in neonates improved also the prognosis of patients with this heart lesion. HLHS, however, continues to be an unresolved problem from ethical, psychological, social and economical points of view. Opinions regarding the care for these patients depend on economical, political, cultural and religious factors. Based on recent experience it is evident, that HLHS is an operable disease in a child, who without surgery would otherwise die. Approximately 40% of patients with HLHS can live 15 years after surgery. The long-term prognosis remains, however, unclear. Because of the need of demanding care for a patient with HLHS, it is necessary to see also the quality of life and hear the opinion of parents. Psychomotor development and the quality of life may be altered by complications. An individualized approach in fetuses and neonates with HLHS on the basis of prenatal diagnosis, analysis of risk factors, provision of detailed informations to parents and indications for surgery in neonates having chance for an acceptable result is considered to be the most acceptable solution. In the future it will be necessary to concentrate the research on prenatal diagnostics, treatment and postoperative follow-up including psychomotor development and quality of life. In the preparation of legislative, which would reflect more sensitively the contemporary possibilities of medicine and at the same time regard the problems of health care organization, as well as social and economic aspects, narrow interdisciplinary cooperation will be required.

Cardiac Surgical Procedures↗
PubMed2001

[Surgical treatment of hypoplastic left heart syndrome].

Hypoplastic left heart syndrome (HLHS) was previously uniformally fatal within the first month of life. The development of the Norwood stage I operation has afforded new hope to the families of newborn infants with this defect. Recent, modification of the Norwood procedure has improved the surgical results. The one-year survival rate after the Norwood procedure is from 50% to 70% in major institutions. The results of second palliation (hemi-Fontan operation or bidirectional Glenn operation) are nearly acceptable. Recently, fetal echocardiography has allowed early diagnosis of HLHS, and after a prenatal diagnosis of HLHS, couples may be offered termination of the pregnancy. It is necessary to improve the results of the Norwood stage I operation, to save more fetuses and neonates with HLHS.

Humans↗
PubMed2004

New developments in the treatment of hypoplastic left heart syndrome.

Hypoplastic left heart syndrome (HLHS) was once a uniformly fatal disease, accounting the majority of neonatal deaths due to congenital heart defects. Twenty-five years of advances in the surgical and medical management of this disease have resulted in dramatic improvements in survival for these children. The goal for patients with HLHS should be survival of near 100% with a good quality of life. The advances described in this manuscript detail some of the new techniques used in the surgical and medical management. For infants undergoing staged reconstruction, the Norwood procedure is performed in the newborn period, followed by a hemi-Fontan operation at 6 months of age, and a modified Fontan operation at 1 to 2 years of age. A significant recent modification of the Norwood procedure is the placement of a right ventricle to pulmonary artery (RV-PA) conduit instead of a systemic artery to pulmonary shunt (modified Blalock-Taussig or BT shunt) as the source of pulmonary blood flow. Our recent experience with this modification demonstrated an incremental increase in survival, improved postoperative stability, and decreased inter-stage mortality. At catheterization, significant differences in hemodynamic parameters were present that were consistent with improved coronary blood flow, decreased volume load to the single right ventricle, and improved pulmonary artery growth in those patients with the RV-PA conduit. The 3rd stage in the surgical treatment of HLHS is the modified Fontan operation or cavo-pulmonary anastamosis. A new approach for Fontan completion is the placement of a stent covered with a thin layer of Goretex from the inferior vena cava to the hemi-Fontan baffle. This can be performed in the catheterization laboratory, with a decrease in post-procedure pleural effusions, lower mortality, and a shorter length of stay. The future of treatment for HLHS may eventually involve a single open-heart procedure with initial and final interventions being performed in the catheterization laboratory. Some cardiac centers perform transplantation for management of HLHS. Survival following transplantation has improved as advances in the pre- and post-operative management continue, along with new options for immunosuppression. Treatment for HLHS continues to evolve, as ongoing work has resulted in improved short and long-term survival. The future for children with this disease is encouraging as evidenced by the remarkable achievements made to date and the current worldwide interest and study of HLHS.

Cardiac Surgical Procedures↗
PubMedDate not supplied

Hypoplastic left heart syndrome.

Hypoplastic left heart syndrome (HLHS) is a complex combination of cardiac malformations that probably results from multiple developmental errors in the early stages of cardiogenesis and that, if left untreated, invariably proves fatal. A variety of chest radiographic findings are seen in patients with HLHS, including an enlarged cardiac silhouette (notably a prominent right atrium), pulmonary venous hypertension, an atrial septal defect, and valvular stenosis or atresia. The recent evolution of palliative surgical procedures (modified Norwood procedure, bidirectional cavopulmonary shunt, modified Fontan procedure, aortic valvuloplasty, heart transplantation) has increased the survival rate in children with HLHS. Echocardiography allows accurate assessment of the size and location of the ductus arteriosus, the hemodynamics of the aortic root, the patency and size of the foramen ovale or atrial septal defect, and the presence of a ventricular septal defect to help determine whether surgical intervention is appropriate and, if so, to facilitate planning. Pediatric radiologists now view radiologic images obtained in patients with HLHS before surgical intervention and at important intervals during treatment. Familiarity with the malformations that characterize HLHS and the surgical procedures used to enhance postnatal survival will help pediatric radiologists provide better care for patients with this relatively common pathologic condition.

Coronary Angiography↗
PubMed2004

[Hypoplastic left heart syndrome].

Hypoplastic left heart syndrome (HLHS) is one of the last remaining problems in pediatric cardiac surgery, which necessitates a search for new solutions and continues to be a challenge for cardiologists and cardiac surgeons. The identification of HLHS in utero is extremely helpful for prompt, appropriate preoperative management. In addition, infants with prenatal diagnosis had an improvement operative mortality on Norwood operation compared to infants diagnosed postnatally. Outcome following Norwood procedure has improved significantly secondary to modifications in the surgical technique for protection of multiorgans, improved perioperative care, and improved anesthetic management. In particular right ventricular (RV)-pulmonary artery (PA) shunt using non-valved polytetrafluoroethylene (PTFE) graft was applied as a first stage palliation of HLHS to prevent hemodynamic instability associated with a classic Norwood procedure. In the modified Norwood (RV-PA shunt), higher diastolic blood pressures and lower Qp/Qs ratios that are associated with a more stable and efficient systemic circulation. In fact, patients who received a RV-PA shunt, rarely received ventilatory manipulations.

Blood Vessel Prosthesis Implantation↗
PubMed1993

Mesenteric ischemia in hypoplastic left heart syndrome.

Hypoplastic left heart syndrome (HLHS) has been widely viewed as a uniformly fatal form of congenital heart disease. Between January 1984 and December 1990, 387 patients with the diagnosis of HLHS were treated at this institution. Mesenteric ischemia was clinically diagnosed in 31 patients (8% incidence) and confirmed by pathology or surgery in 25 of those patients. The mean age at the time of onset was 17.5 +/- 5.4 weeks and only 13% were premature newborns. In 80% of the patients a low perfusion state and significant hypotension were documented within 48 hours prior to the diagnosis of bowel ischemia. Nine patients (29%) required operative intervention (bowel resection 4, diffuse ischemia 3, and simple drainage 2). Overall, at operation or at autopsy, nine patients (29%) had diffuse gastrointestinal ischemia. Of 31 patients with mesenteric ischemia, 26 children (84%) died shortly after onset of the gastrointestinal symptoms regardless of means of management. Five patients (10%) initially improved with aggressive medical and/or surgical management; however, 4 subsequently died secondary to complications of their primary cardiac disease. Therefore, the overall mortality of patients with mesenteric ischemia was 97%. Previous reports have estimated that up to 7% of full-term newborns with symptomatic congenital heart disease may develop necrotizing enterocolitis (NEC). Our unique group of patients with HLHS is comprised mostly of full-term infants who developed onset of mesenteric ischemia at a mean age of 4 months associated with an underlying low perfusion state. This mesenteric ischemia has been erroneously identified as NEC.(ABSTRACT TRUNCATED AT 250 WORDS)

Diagnosis, Differential↗
PubMed1989

Hypoplastic left heart syndrome.

Hypoplastic left heart syndrome is a common lethal congenital cardiac malformation. The anatomy and physiology dictate therapy in the newborn period. Two stages of reconstructive surgical management are detailed.

Heart Defects, Congenital↗
PubMed1993

Care of infants with hypoplastic left heart syndrome.

Hypoplastic left heart syndrome (HLHS) is a common congenital heart defect that is universally fatal without surgical intervention. Two distinctly different surgical options, heart transplantation and reconstruction surgery, have been successful in treating infants with HLHS. This article reviews the anatomy and pathophysiology of HLHS. Preoperative care of infants with HLHS is discussed. The care required by infants with HLHS after heart transplantation is the same as that required by other infants who need heart transplant and has been reported. The primary focus of this report is postoperative care after staged reconstructive surgery for infants with HLHS.

Blood Circulation↗
PubMed2004

Morphometry of coronary capillaries in hypoplastic left heart syndrome.

BACKGROUND: Hypoplastic left heart syndrome is a condition characterized by a constellation of morphological malformations affecting the left side of the heart. We studied the capillary network, and quantified the capillarization of the ventricular myocardium, which, if different from normal, may have implications for the success of surgical reconstruction. METHODS: The capillaries were detected by immunohistochemistry using a monoclonal antibody (von Willebrand's factor) against the endothelium. Hearts with hypoplastic left heart syndrome have higher mean and maximal diffusion distances from any arbitrary point to the nearest capillary than normal hearts. RESULTS: There was no significant difference in the heterogeneity of capillary distribution between the hearts with hypoplastic left heart syndrome and the control heart. Increase in distance was found in both the right and left ventricles. CONCLUSIONS: Hearts with hypoplastic left heart syndrome show a reduction in the capillarization of both the right and left ventricles compared with age-matched controls. We believe this may be an inherent abnormality of hypoplastic left heart syndrome that may have implications for ventricular development.

Capillaries↗
PubMed2006

The nomenclature, definition and classification of hypoplastic left heart syndrome.

The hypoplastic left heart syndrome encompasses a spectrum of cardiac malformations that are characterized by significant underdevelopment of the components of the left heart and the aorta, including the left ventricular cavity and mass. At the severe end of the spectrum is found the combination of aortic and mitral atresia, when the left ventricle can be close to non-existent. At the mild end are the patients with hypoplasia of the aortic and mitral valves, but without intrinsic valvar stenosis or atresia, and milder degrees of left ventricular hypoplasia. Although the majority of the patients are suitable only for functionally univentricular repair, a small minority may be candidates for biventricular repair. The nature of the syndrome was a topic for discussion at the second meeting of the International Working Group for Mapping and Coding of Nomenclatures for Paediatric and Congenital Heart Disease, the Nomenclature Working Group, held in Montreal, Canada, over the period January 17 through 19, 2003. Subsequent to these discussions, the Nomenclature Working Group was able to create a bidirectional crossmap between the nomenclature initially produced jointly on behalf of the European Association for Cardio-Thoracic Surgery and the Society of Thoracic Surgeons, and the alternative nomenclature developed on behalf of the Association for European Paediatric Cardiology. This process is a part of the overall efforts of the Nomenclature Working Group to create a comprehensive and all-inclusive international system of nomenclature for paediatric and congenital cardiac disease, the International Paediatric and Congenital Cardiac Code. In this review, we discuss the evolution of nomenclature and surgical treatment for the spectrum of lesions making up the hypoplastic left heart syndrome and its related malformations. We also present the crossmap of the associated terms for diagnoses and procedures, as recently completed by the Nomenclature Working Group.

Diagnosis, Differential↗