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Pediatric Cardiology Oct 2011Aortic atresia-mitral stenosis (AA-MS) has been implicated as a determinant of outcome after Stage-1 palliation (S1P) in hypoplastic left heart syndrome (HLHS).Studies... (Comparative Study)
Comparative Study
Hypoplastic left heart syndrome and aortic atresia-mitral stenosis variant: role of myocardial protection strategy and impact of ventriculo-coronary connections after stage I palliation.
Aortic atresia-mitral stenosis (AA-MS) has been implicated as a determinant of outcome after Stage-1 palliation (S1P) in hypoplastic left heart syndrome (HLHS).Studies evaluating the association of AA-MS with ventriculo-coronary connections (VCC) and mortality report conflicting results. The significance of VCC, myocardial protection, and shunt strategy after S1P has yet to be determined. Between January 2005 and July 2009, 100 neonates with HLHS underwent S1P. Mitral and aortic valves and presence of VCC were assessed. Antegrade continuous cold blood cardioplegia was administered throughout the vast extent of the neo-aortic reconstruction. A right ventricle-to-pulmonary shunt was used for an ascending aortic diameter of 0.6 mm/kg or less. Survival analysis was performed to determine predictors and assess impact of AA-MS and VCC on hospital and interstage mortality. Twenty-seven (of 100) patients had AA-MS. The mean age and weight at S1P were 6.5 ± 2.8 days and 3.09 ± 0.47 kg, respectively. VCC were found in 56% of AA-MS. Twenty-two had Norwood-Sano, 3 had classic Norwood, and 2 had hybrid S1P. VCC were associated with AA-MS, endocardial fibroelastosis, and ascending aortic size <2 mm (P < 0.05) but not higher mortality (P = ns). Operative and interstage survival for AA-MS after S1P was 85.2 and 71%, respectively (not statistically different compared to all other subtypes; P = ns). Actuarial survival after S1P at 1, 3, 6, 12, and 36 months was 92.9 ± 4.9, 78.6 ± 7.8, 75 ± 8.2, 71.3 ± 8.3, and 71.3 ± 8.3%, respectively. Intact atrial septum and post-S1P renal dysfunction (P < 0.05) were independent predictors of hospital and interstage mortality. In patients with HLHS, AA-MS carries no survival disadvantage after S1P during the hospital and interstage period regardless of VCC. Intact atrial septum and post-S1P renal dysfunction predict early and interstage mortality. Myocardial protection and shunt strategy might influence the outcome in this HLHS variant.
Topics: Abnormalities, Multiple; Aorta; Aortic Valve; Aortic Valve Stenosis; Cardiac Surgical Procedures; Echocardiography; Female; Follow-Up Studies; Hospital Mortality; Humans; Hypoplastic Left Heart Syndrome; Infant, Newborn; Male; Mitral Valve; Mitral Valve Stenosis; Palliative Care; Retrospective Studies; Survival Rate; Time Factors; Treatment Outcome; United States
PubMed: 21644013
DOI: 10.1007/s00246-011-0017-6 -
Anales Espanoles de Pediatria Jan 1986We present the pathological study of 17 cases of mitral atresia with patent aortic valve and their surgical implications. The study is based on the type of...
We present the pathological study of 17 cases of mitral atresia with patent aortic valve and their surgical implications. The study is based on the type of atrioventricular connection, presence or absence of ventricular septum, size of the left ventricle and relation of the aorta with the aforementioned ventricle. We have classified mitral atresia into two groups by assessing these data. Group A) Both ventricles and atrioventricular connections are present and there is ventricular septal defect. The aorta may connect with an either normal or hypoplastic left ventricle or it may emerge in double outlet from the right ventricle. Group B) Absent left ventricle and atrioventricular connection. The aorta emerges from the single ventricle or the rudimentary chamber. Thirteen cases belonged to group A and four to group B. The pathological structure of mitral atresia requires the decompression of the left atrium by atrioseptostomy during the neonatal period to enable survival. On a second stage a palliative correction should be carried out, which; depending on ventricular size and outlet of the vessels, will be a valvular prosthesis left atrium-left ventricle or the modified atriopulmonary technique connecting the new left atrium with the systemic ventricle and aorta after resecting the auricular septum and connecting the right atrium with the pulmonary artery.
Topics: Female; Heart Ventricles; Humans; Infant, Newborn; Male; Mitral Valve
PubMed: 3963641
DOI: No ID Found -
Journal of Pediatric Surgery Sep 2004Biliary atresia is a cholestatic disorder of infancy that is associated with other anatomic anomalies in approximately 20% of cases. These frequently are defects in... (Review)
Review
Biliary atresia is a cholestatic disorder of infancy that is associated with other anatomic anomalies in approximately 20% of cases. These frequently are defects in situs determination and laterality, causing syndromes of heterotaxy and complex heart defects. The authors describe an infant with both biliary atresia and hypoplastic left heart syndrome (HLHS) in the absence of a laterality defect or evidence of any other morphologic defect. The presence of a hypoplastic left ventricle in a patient with biliary atresia has previously been reported only in the context of a heterotaxy syndrome. The coexistence of these 2 disorders raises etiologic considerations and represents a potential challenge for the surgical treatment of both conditions.
Topics: Abnormalities, Multiple; Aortic Valve; Biliary Atresia; Fontan Procedure; Humans; Hypoplastic Left Heart Syndrome; Infant, Newborn; Jaundice, Neonatal; Laparotomy; Male; Mitral Valve; Palliative Care; Portoenterostomy, Hepatic; Ultrasonography, Prenatal
PubMed: 15359401
DOI: 10.1016/j.jpedsurg.2004.05.031 -
Journal of Cardiography Dec 1986Two-dimensional echocardiographic examinations were performed for 86 patients with the hypoplastic right heart syndrome (55 cases of tricuspid atresia, 9 of pure...
Two-dimensional echocardiographic examinations were performed for 86 patients with the hypoplastic right heart syndrome (55 cases of tricuspid atresia, 9 of pure pulmonary stenosis and 22 of pure pulmonary atresia). Prolapse of the anterior mitral leaflet was detected in 14 patients (16.3%). Four of these 14 patients had mitral regurgitation. Among the patients with mitral valve prolapse, ruptured chordae tendineae of the mitral valve were detected in two, and elongation of the chordae in one. There were no significant relationships between the incidence of mitral valve prolapse and sex, age, type of tricuspid atresia, previous palliative surgery, or PaO2. However, one patient with pure pulmonary stenosis, who had had mitral valve prolapse without mitral regurgitation, developed mitral regurgitation several months after undergoing a Blalock-Taussig shunt operation. The mechanism responsible for mitral valve prolapse in the hypoplastic right heart syndrome is not clear. Multiple factors, such as long-standing left ventricular volume overload, hypoxic myocardial damage, and anatomical chordal abnormalities may cause mitral valve prolapse. The presence of a mitral valve abnormality has an important bearing on the natural history as well as the surgical procedure of choice. The need for careful echocardiographic examinations to detect mitral valve abnormalities in the hypoplastic right heart syndrome is thus underscored.
Topics: Adolescent; Adult; Child; Child, Preschool; Echocardiography; Female; Humans; Infant; Infant, Newborn; Male; Mitral Valve Insufficiency; Mitral Valve Prolapse; Pulmonary Valve; Pulmonary Valve Stenosis; Tricuspid Valve
PubMed: 3429912
DOI: No ID Found -
Cardiology in the Young Feb 2006
Review
Topics: Echocardiography; Heart Ventricles; Humans; Mitral Valve; Tricuspid Atresia
PubMed: 16401360
DOI: 10.1017/S1047951105002295 -
The Annals of Thoracic Surgery Apr 1980A 13-year-old patient with visceral and atrial situs solitus, dextrocardia, dextro (D) transposition of the great vessels with subaortic conus, tricuspid atresia,...
A 13-year-old patient with visceral and atrial situs solitus, dextrocardia, dextro (D) transposition of the great vessels with subaortic conus, tricuspid atresia, massive mitral valve insufficiency, and previously performed pulmonary artery banding was seen at our hospital. A right atrial to pulmonary artery conduit procedure and mitral valve replacement successfully repaired this child's circulation.
Topics: Adolescent; Bioprosthesis; Blood Vessel Prosthesis; Cardiac Catheterization; Dextrocardia; Heart Valve Prosthesis; Hemodynamics; Humans; Male; Mitral Valve Insufficiency; Pulmonary Artery; Transposition of Great Vessels; Tricuspid Valve
PubMed: 7362331
DOI: 10.1016/s0003-4975(10)61488-9 -
Journal of the American Heart... Apr 2022Background Hypoplastic left heart syndrome is associated with significant morbidity and mortality. We aimed to assess the influence of left ventricular morphology and...
Background Hypoplastic left heart syndrome is associated with significant morbidity and mortality. We aimed to assess the influence of left ventricular morphology and choice of shunt on adverse outcome in patients with hypoplastic left heart syndrome and stage 1 palliation. Methods and Results This was a retrospective analysis of patients with hypoplastic left heart syndrome with stage 1 palliation between 1999 and 2018 in Sweden. Patients (n=167) were grouped based on the anatomic subtypes aortic-mitral atresia, aortic atresia-mitral stenosis (AA-MS), and aortic-mitral stenosis. The left ventricular phenotypes including globular left ventricle (Glob-LV), miniaturized and slit-like left ventricle (LV), and the incidence of major adverse events (MAEs) including mortality were assessed. The overall mortality and MAEs were 31% and 41%, respectively. AA-MS (35%) was associated with both mortality (all other subtypes versus AA-MS: interstage-I: hazard ratio [HR], 2.7; =0.006; overall: HR, 2.2; =0.005) and MAEs (HR, 2.4; =0.0009). Glob-LV (57%), noticed in all patients with AA-MS, 61% of patients with aortic stenosis-mitral stenosis, and 19% of patients with aortic atresia-mitral atresia, was associated with both mortality (all other left ventricular phenotypes versus Glob-LV: interstage-I: HR, 4.5; =0.004; overall: HR, 3.4; =0.0007) and MAEs (HR, 2.7; =0.0007). There was no difference in mortality and MAEs between patients with AA-MS and without AA-MS with Glob-LV (>0.15). Patients with AA-MS (35%) or Glob-LV (38%) palliated with a Blalock-Taussig shunt had higher overall mortality compared with those palliated with Sano shunts, irrespective of the stage 1 palliation year (AA-MS: HR, 2.6; =0.04; Glob- LV: HR, 2.1; =0.03). Conclusions Glob-LV and AA-MS are independent morphological risk factors for adverse short- and long- term outcome, especially if a Blalock-Taussig shunt is used as part of stage 1 palliation. These findings are important for the clinical management of patients with hypoplastic left heart syndrome.
Topics: Heart Ventricles; Humans; Hypoplastic Left Heart Syndrome; Mitral Valve Stenosis; Palliative Care; Retrospective Studies; Sweden; Treatment Outcome
PubMed: 35348003
DOI: 10.1161/JAHA.121.022929 -
Pediatrics Mar 1960
Topics: Aortic Valve; Heart Defects, Congenital; Humans; Mitral Valve
PubMed: 13843122
DOI: No ID Found -
British Heart Journal Sep 1969
Topics: Child; Female; Heart Atria; Heart Diseases; Heart Septal Defects, Ventricular; Humans; Mitral Valve; Pulmonary Embolism; Thrombosis; Transposition of Great Vessels; Tricuspid Valve
PubMed: 5351304
DOI: 10.1136/hrt.31.5.649 -
World Journal For Pediatric &... Sep 2022This manuscript will provide information about hypoplastic left heart syndrome (HLHS) and related malformations, including definitions, morphology, and classification,...
This manuscript will provide information about hypoplastic left heart syndrome (HLHS) and related malformations, including definitions, morphology, and classification, based on the 2021 (IPCCC) and the (ICD-11). HLHS is defined as "a spectrum of congenital cardiovascular malformations with normally aligned great arteries without a common atrioventricular junction, characterized by underdevelopment of the left heart with significant hypoplasia of the left ventricle including atresia, stenosis, or hypoplasia of the aortic or mitral valve, or both valves, and hypoplasia of the ascending aorta and aortic arch." Functionally univentricular heart is defined as "a spectrum of congenital cardiac malformations in which the ventricular mass may not readily lend itself to partitioning that commits one ventricular pump to the systemic circulation, and another to the pulmonary circulation." The Norwood operation is synonymous with the term "Norwood (Stage 1)" and is defined as (1) creation of an aortopulmonary connection and neoaortic arch construction resulting in univentricular physiology and (2) creation of a controlled source of pulmonary blood flow with a calibrated systemic-to-pulmonary artery shunt, a right ventricle to pulmonary artery conduit, or rarely, a cavopulmonary connection. The goals of the Norwood (Stage 1) Operation are creation of (1) unobstructed systemic blood flow via aortopulmonary connection and neoaortic arch construction, (2) unobstructed coronary blood flow, (3) unobstructed flow across the atrial septum, and (4) controlled pulmonary blood flow.
Topics: Child; Heart Defects, Congenital; Heart Ventricles; Humans; Hypoplastic Left Heart Syndrome; Norwood Procedures; Pulmonary Artery
PubMed: 36053108
DOI: 10.1177/21501351221114770