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[PMID]:28977206
[Au] Autor:Pillai VV; Karunakaran J
[Ad] Endereço:Sree Chitra Tirunal Institute for Medical Sciences and Technology, Department of Cardiovascular and Thoracic Surgery, Trivandrum, Kerala, India.
[Ti] Título:Repair of Double Orifice Left AV Valve (DOLAVV) with Endocardial Cushion Defect in Adult.
[So] Source:Braz J Cardiovasc Surg;32(4):338-340, 2017 Jul-Aug.
[Is] ISSN:1678-9741
[Cp] País de publicação:Brazil
[La] Idioma:eng
[Ab] Resumo:Double orifice left atrioventricular valve (DOLAVV) or double orifice mitral valve (DOMV) is a rare congenital cardiac anomaly manifesting either as an isolated lesion (mitral stenosis or mitral insufficiency) or in association with other congenital cardiac defects. Signs of mitral valve disease are usually present along with the symptoms of associated coexistent congenital heart diseases. Mitral insufficiency due to annular dilatation is seen when DOLAVV is associated with endocardial cushion defects. Surgical intervention like mitral valve repair or replacement is required in 50% of patients and yields good results. We report a case of a 56-year-old lady who successfully underwent surgical correction of DOLAVV with partial atrioventricular canal defect.
[Mh] Termos MeSH primário: Comunicação Atrioventricular/cirurgia
Defeitos dos Septos Cardíacos/cirurgia
Valva Mitral/anormalidades
[Mh] Termos MeSH secundário: Comunicação Atrioventricular/complicações
Feminino
Defeitos dos Septos Cardíacos/complicações
Implante de Prótese de Valva Cardíaca/métodos
Seres Humanos
Meia-Idade
Valva Mitral/cirurgia
Anuloplastia da Valva Mitral/métodos
Resultado do Tratamento
[Pt] Tipo de publicação:CASE REPORTS
[Em] Mês de entrada:1710
[Cu] Atualização por classe:171023
[Lr] Data última revisão:
171023
[Sb] Subgrupo de revista:IM
[Da] Data de entrada para processamento:171005
[St] Status:MEDLINE


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[PMID]:28211263
[Au] Autor:Ford SM; McPheeters MT; Wang YT; Ma P; Gu S; Strainic J; Snyder C; Rollins AM; Watanabe M; Jenkins MW
[Ad] Endereço:Rainbow Babies and Children's Hospital Division of Neonatology, University Hospitals, Cleveland, Ohio, USA.
[Ti] Título:Increased regurgitant flow causes endocardial cushion defects in an avian embryonic model of congenital heart disease.
[So] Source:Congenit Heart Dis;12(3):322-331, 2017 May.
[Is] ISSN:1747-0803
[Cp] País de publicação:United States
[La] Idioma:eng
[Ab] Resumo:BACKGROUND: The relationship between changes in endocardial cushion and resultant congenital heart diseases (CHD) has yet to be established. It has been shown that increased regurgitant flow early in embryonic heart development leads to endocardial cushion defects, but it remains unclear how abnormal endocardial cushions during the looping stages might affect the fully septated heart. The goal of this study was to reproducibly alter blood flow in vivo and then quantify the resultant effects on morphology of endocardial cushions in the looping heart and on CHDs in the septated heart. METHODS: Optical pacing was applied to create regurgitant flow in embryonic hearts, and optical coherence tomography (OCT) was utilized to quantify regurgitation and morphology. Embryonic quail hearts were optically paced at 3 Hz (180 bpm, well above intrinsic rate 60-110 bpm) at stage 13 of development (3-4 weeks human) for 5 min. Pacing fatigued the heart and led to at least 1 h of increased regurgitant flow. Resultant morphological changes were quantified with OCT imaging at stage 19 (cardiac looping-4-5 weeks human) or stage 35 (4 chambered heart-8 weeks human). RESULTS: All paced embryos imaged at stage 19 displayed structural changes in cardiac cushions. The amount of regurgitant flow immediately after pacing was inversely correlated with cardiac cushion size 24-h post pacing (P value < .01). The embryos with the most regurgitant flow and smallest cushions after pacing had a decreased survival rate at 8 days (P < .05), indicating that those most severe endocardial cushion defects were lethal. Of the embryos that survived to stage 35, 17/18 exhibited CHDs including valve defects, ventricular septal defects, hypoplastic ventricles, and common AV canal. CONCLUSION: The data illustrate a strong inverse relationship in which regurgitant flow precedes abnormal and smaller cardiac cushions, resulting in the development of CHDs.
[Mh] Termos MeSH primário: Velocidade do Fluxo Sanguíneo/fisiologia
Comunicação Atrioventricular/etiologia
Cardiopatias Congênitas/embriologia
[Mh] Termos MeSH secundário: Animais
Modelos Animais de Doenças
Comunicação Atrioventricular/diagnóstico
Comunicação Atrioventricular/embriologia
Cardiopatias Congênitas/complicações
Cardiopatias Congênitas/fisiopatologia
Organogênese
Codorniz
Tomografia de Coerência Óptica
[Pt] Tipo de publicação:JOURNAL ARTICLE
[Em] Mês de entrada:1711
[Cu] Atualização por classe:171102
[Lr] Data última revisão:
171102
[Sb] Subgrupo de revista:IM
[Da] Data de entrada para processamento:170218
[St] Status:MEDLINE
[do] DOI:10.1111/chd.12443


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[PMID]:27542407
[Au] Autor:Ma P; Gu S; Karunamuni GH; Jenkins MW; Watanabe M; Rollins AM
[Ad] Endereço:Department of Biomedical Engineering, Case Western Reserve University, Cleveland, Ohio; and.
[Ti] Título:Cardiac neural crest ablation results in early endocardial cushion and hemodynamic flow abnormalities.
[So] Source:Am J Physiol Heart Circ Physiol;311(5):H1150-H1159, 2016 Nov 01.
[Is] ISSN:1522-1539
[Cp] País de publicação:United States
[La] Idioma:eng
[Ab] Resumo:Cardiac neural crest cell (CNCC) ablation creates congenital heart defects (CHDs) that resemble those observed in many syndromes with craniofacial and cardiac consequences. The loss of CNCCs causes a variety of great vessel defects, including persistent truncus arteriosus and double-outlet right ventricle. However, because of the lack of quantitative volumetric measurements, less severe defects, such as great vessel size changes and valve defects, have not been assessed. Also poorly understood is the role of abnormal cardiac function in the progression of CNCC-related CHDs. CNCC ablation was previously reported to cause abnormal cardiac function in early cardiogenesis, before the CNCCs arrive in the outflow region of the heart. However, the affected functional parameters and how they correlate with the structural abnormalities were not fully characterized. In this study, using a CNCC-ablated quail model, we contribute quantitative phenotyping of CNCC ablation-related CHDs and investigate abnormal early cardiac function, which potentially contributes to late-stage CHDs. Optical coherence tomography was used to assay early- and late-stage embryos and hearts. In CNCC-ablated embryos at four-chambered heart stages, great vessel diameter and left atrioventricular valve leaflet volumes are reduced. Earlier, at cardiac looping stages, CNCC-ablated embryos exhibit abnormally twisted bodies, abnormal blood flow waveforms, increased retrograde flow percentage, and abnormal cardiac cushions. The phenotypes observed in this CNCC-ablation model were also strikingly similar to those found in an established avian fetal alcohol syndrome model, supporting the contribution of CNCC dysfunction to the development of alcohol-induced CHDs.
[Mh] Termos MeSH primário: Comunicação Atrioventricular/embriologia
Coração/embriologia
Crista Neural/cirurgia
[Mh] Termos MeSH secundário: Animais
Aorta/anormalidades
Aorta/diagnóstico por imagem
Aorta/embriologia
Embrião não Mamífero
Comunicação Atrioventricular/diagnóstico por imagem
Transtornos do Espectro Alcoólico Fetal
Coração/diagnóstico por imagem
Cardiopatias Congênitas/diagnóstico por imagem
Cardiopatias Congênitas/embriologia
Valvas Cardíacas/anormalidades
Valvas Cardíacas/diagnóstico por imagem
Valvas Cardíacas/embriologia
Terapia a Laser
Crista Neural/embriologia
Tamanho do Órgão
Fenótipo
Artéria Pulmonar/anormalidades
Artéria Pulmonar/diagnóstico por imagem
Artéria Pulmonar/embriologia
Codorniz
Tomografia de Coerência Óptica
[Pt] Tipo de publicação:JOURNAL ARTICLE
[Em] Mês de entrada:1706
[Cu] Atualização por classe:171101
[Lr] Data última revisão:
171101
[Sb] Subgrupo de revista:IM
[Da] Data de entrada para processamento:160821
[St] Status:MEDLINE
[do] DOI:10.1152/ajpheart.00188.2016


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[PMID]:26238793
[Au] Autor:Davey BT; Rychik J
[Ad] Endereço:The Fetal Heart Program, The Children's Hospital of Philadelphia, 34th Street and Civic Center Blvd., Philadelphia, PA, 19104, USA.
[Ti] Título:The Natural History of Atrioventricular Valve Regurgitation Throughout Fetal Life in Patients with Atrioventricular Canal Defects.
[So] Source:Pediatr Cardiol;37(1):50-4, 2016 Jan.
[Is] ISSN:1432-1971
[Cp] País de publicação:United States
[La] Idioma:eng
[Ab] Resumo:Atrioventricular valve regurgitation (AVVR) influences morbidity and mortality in the atrioventricular canal defect (AVC). Fetal cardiac structures are subject to hemodynamic changes, as well as growth and maturation during gestation, which may alter the degree of AVVR and affect prognosis. We sought to investigate the frequency of change in degree of AVVR documented by fetal echocardiography (echo) between different periods of gestational age. Subjects with AVC seen in the Fetal Heart Program between January 2008 and September 2010 were identified. Degree of AVVR was assessed by color Doppler imaging and categorized as Grade 0 (no AVVR), Grade 1 (hemodynamically insignificant AVVR = trivial or mild), and Grade 2 (hemodynamically important AVVR = ≥moderate). Levels of AVVR between periods were compared. Forty-three fetuses were analyzed. Overall, 60% had no change, 14% had a decrease, and 26% had an increase in AVVR grade. Two fetuses progressed from Grade 0 or 1 to Grade 2, while one fetus decreased from Grade 2 to Grade 0. Trisomy 21 and heterotaxy syndrome were not risk factors for AVVR progression. Transitional and incomplete canal defects may be more susceptible to AVVR progression. Sixty percent of fetuses with AVC will not exhibit progression of AVVR between the second and third trimesters of gestation. In those who exhibit change, it is most often within a hemodynamically insignificant range between none and mild regurgitation (Grades 0 and 1). These findings have implications for the counseling, follow-up, and delivery plans of the fetus with AVC defect.
[Mh] Termos MeSH primário: Comunicação Atrioventricular/diagnóstico por imagem
Coração Fetal/diagnóstico por imagem
Defeitos dos Septos Cardíacos/diagnóstico por imagem
Valvas Cardíacas/diagnóstico por imagem
[Mh] Termos MeSH secundário: Ecocardiografia
Comunicação Atrioventricular/fisiopatologia
Feminino
Coração Fetal/fisiopatologia
Feto
Idade Gestacional
Defeitos dos Septos Cardíacos/fisiopatologia
Valvas Cardíacas/fisiopatologia
Hemodinâmica
Seres Humanos
Gravidez
Prognóstico
Estudos Retrospectivos
Ultrassonografia Doppler em Cores
[Pt] Tipo de publicação:JOURNAL ARTICLE; RESEARCH SUPPORT, NON-U.S. GOV'T
[Em] Mês de entrada:1611
[Cu] Atualização por classe:170922
[Lr] Data última revisão:
170922
[Sb] Subgrupo de revista:IM
[Da] Data de entrada para processamento:150805
[St] Status:MEDLINE
[do] DOI:10.1007/s00246-015-1237-y


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[PMID]:26460913
[Au] Autor:Phillips C; Boyd M
[Ti] Título:Relationship-Based Care for Newborns With Down Syndrome and Endocardial Cushion Defect.
[So] Source:Nurs Womens Health;19(5):410-21, 2015 Oct-Nov.
[Is] ISSN:1751-486X
[Cp] País de publicação:United States
[La] Idioma:eng
[Ab] Resumo:Down syndrome with endocardial cushion defect is a challenging diagnosis for parents as well as members of the health care team. Utilizing a framework of relationship-based care, nurses are in a position to positively affect parents' experience by providing education, advocacy, and support from initial diagnosis through discharge. The plan of care is multidisciplinary and focuses on critical developmental needs, such as bonding and feeding. Because Down syndrome is associated with multiple anomalies, anticipatory guidance is needed to assist parents with establishing a health maintenance plan for their child after discharge.
[Mh] Termos MeSH primário: Síndrome de Down/psicologia
Comunicação Atrioventricular/enfermagem
Relações Enfermeiro-Paciente
Pais/educação
[Mh] Termos MeSH secundário: Síndrome de Down/enfermagem
Comunicação Atrioventricular/diagnóstico
Comunicação Atrioventricular/terapia
Seres Humanos
Pais/psicologia
Apoio Social
[Pt] Tipo de publicação:JOURNAL ARTICLE
[Em] Mês de entrada:1702
[Cu] Atualização por classe:170206
[Lr] Data última revisão:
170206
[Sb] Subgrupo de revista:N
[Da] Data de entrada para processamento:151014
[St] Status:MEDLINE
[do] DOI:10.1111/1751-486X.12232


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[PMID]:26354652
[Au] Autor:Jonker V; Atallah J; Horne D; Rebeyka IM
[Ad] Endereço:Division of Cardiac Surgery, Department of Surgery, University of Alberta, Edmonton, Alberta, Canada.
[Ti] Título:A Novel Surgical Technique for Repair of Congenitally Corrected Transposition of the Great Arteries With Atrioventricular Septal Defect: Avoiding Damage to the Conduction System.
[So] Source:Ann Thorac Surg;100(3):1121-3, 2015 Sep.
[Is] ISSN:1552-6259
[Cp] País de publicação:Netherlands
[La] Idioma:eng
[Ab] Resumo:We present a description of the surgical repair of the unusual anatomy of congenitally corrected transposition of the great arteries (S, L, L) and balanced atrioventricular septal defect. This anatomy not only presents a dilemma regarding palliation versus anatomic correction, but also regarding the approach to the conduction tissue during surgical repair.
[Mh] Termos MeSH primário: Defeitos dos Septos Cardíacos/cirurgia
Transposição dos Grandes Vasos/cirurgia
[Mh] Termos MeSH secundário: Arritmias Cardíacas
Síndrome de Brugada
Doença do Sistema de Condução Cardíaco
Procedimentos Cirúrgicos Cardíacos/métodos
Comunicação Atrioventricular
Feminino
Sistema de Condução Cardíaco/anormalidades
Defeitos dos Septos Cardíacos/complicações
Seres Humanos
Recém-Nascido
Transposição dos Grandes Vasos/complicações
[Pt] Tipo de publicação:CASE REPORTS; JOURNAL ARTICLE
[Em] Mês de entrada:1512
[Cu] Atualização por classe:171116
[Lr] Data última revisão:
171116
[Sb] Subgrupo de revista:AIM; IM
[Da] Data de entrada para processamento:150911
[St] Status:MEDLINE


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[PMID]:25893250
[Au] Autor:Zhao CM; Peng LY; Li L; Liu XY; Wang J; Zhang XL; Yuan F; Li RG; Qiu XB; Yang YQ
[Ad] Endereço:Department of Cardiology, Tongji Hospital, Tongji University School of Medicine, Shanghai, China; Division of Medical Genetics, Tongji University School of Medicine, Shanghai, China.
[Ti] Título:PITX2 Loss-of-Function Mutation Contributes to Congenital Endocardial Cushion Defect and Axenfeld-Rieger Syndrome.
[So] Source:PLoS One;10(4):e0124409, 2015.
[Is] ISSN:1932-6203
[Cp] País de publicação:United States
[La] Idioma:eng
[Ab] Resumo:Congenital heart disease (CHD), the most common type of birth defect, is still the leading non-infectious cause of infant morbidity and mortality in humans. Aggregating evidence demonstrates that genetic defects are involved in the pathogenesis of CHD. However, CHD is genetically heterogeneous and the genetic components underpinning CHD in an overwhelming majority of patients remain unclear. In the present study, the coding exons and flanking introns of the PITX2 gene, which encodes a paired-like homeodomain transcription factor 2essential for cardiovascular morphogenesis as well as maxillary facial development, was sequenced in 196 unrelated patients with CHD and subsequently in the mutation carrier's family members available. As a result, a novel heterozygous PITX2 mutation, p.Q102X for PITX2a, or p.Q148X for PITX2b, or p.Q155X for PITX2c, was identified in a family with endocardial cushion defect (ECD) and Axenfeld-Rieger syndrome (ARS). Genetic analysis of the pedigree showed that the nonsense mutation co-segregated with ECD and ARS transmitted in an autosomal dominant pattern with complete penetrance. The mutation was absent in 800 control chromosomes from an ethnically matched population. Functional analysis by using a dual-luciferase reporter assay system revealed that the mutant PITX2 had no transcriptional activity and that the mutation eliminated synergistic transcriptional activation between PITX2 and NKX2.5, another transcription factor pivotal for cardiogenesis. To our knowledge, this is the first report on the association of PITX2 loss-of-function mutation with increased susceptibility to ECD and ARS. The findings provide novel insight into the molecular mechanisms underpinning ECD and ARS, suggesting the potential implications for the antenatal prophylaxis and personalized treatment of CHD and ARS.
[Mh] Termos MeSH primário: Segmento Anterior do Olho/anormalidades
Comunicação Atrioventricular/genética
Anormalidades do Olho/genética
Proteínas de Homeodomínio/genética
Mutação
Fatores de Transcrição/genética
[Mh] Termos MeSH secundário: Sequência de Aminoácidos
Animais
Células CHO
Criança
Pré-Escolar
China
Mapeamento Cromossômico
Estudos de Coortes
Cricetulus
Feminino
Genótipo
Heterozigoto
Seres Humanos
Masculino
Dados de Sequência Molecular
Linhagem
Penetrância
Homologia de Sequência de Aminoácidos
Ativação Transcricional
[Pt] Tipo de publicação:JOURNAL ARTICLE; RESEARCH SUPPORT, NON-U.S. GOV'T
[Nm] Nome de substância:
0 (Homeodomain Proteins); 0 (Transcription Factors); 184787-43-7 (homeobox protein PITX2)
[Em] Mês de entrada:1602
[Cu] Atualização por classe:170220
[Lr] Data última revisão:
170220
[Sb] Subgrupo de revista:IM
[Da] Data de entrada para processamento:150421
[St] Status:MEDLINE
[do] DOI:10.1371/journal.pone.0124409


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[PMID]:25886812
[Au] Autor:Stephens EH; Ibrahimiye AN; Yerebakan H; Yilmaz B; Chelliah A; Levasseur S; Mosca RS; Chen JM; Chai P; Quaegebeur J; Bacha EA
[Ad] Endereço:Cardiac, Thoracic, and Vascular Surgery, Columbia University Medical Center, New York, New York.
[Ti] Título:Early Complete Atrioventricular Canal Repair Yields Outcomes Equivalent to Late Repair.
[So] Source:Ann Thorac Surg;99(6):2109-15; discussion 2115-6, 2015 Jun.
[Is] ISSN:1552-6259
[Cp] País de publicação:Netherlands
[La] Idioma:eng
[Ab] Resumo:BACKGROUND: Repair of complete atrioventricular canal early in infancy has traditionally carried greater morbidity and mortality than repair performed later. However, an individualized anatomy-based repair may give young infants outcomes that are equivalent to older patients. METHODS: We retrospectively reviewed 139 patients who underwent complete atrioventricular canal repair from January 2005 to December 2012. An individualized approach was used: 2-patch repair was performed in 98 patients for large ventricular septal defects and a modified single-patch ("Australian technique") was used in 41 for "shallow" ventricular septal defects. RESULTS: The average age was 25.5 ± 3.9 weeks, 50% were boys, and 78% had trisomy 21. Mean follow-up was 5.1 ± 0.2 years, with 100% completeness of data. There were 3 in-hospital deaths (2.1%) and 1 late death (0.7%). A permanent pacemaker was required in 2 patients (1.4%). The rate for left atrioventricular valve reoperation was 8% at a mean of 211 ± 238 days after the original repair (range, 6 to 682 days). Compared with patients aged older than 3 months, the 39 patients (28%) who were younger than 3 months had similar perioperative courses and rate of reoperation. Compared with patients with an Australian repair, the 98 patients (71%) with a 2-patch repair were more likely to have trisomy 21 and had slightly increased cardiopulmonary bypass and cross-clamp times but similar outcomes. Multivariate analysis showed postoperative left atrioventricular valve regurgitation greater than 2 and left ventricular outflow tract obstruction were significant risk factors for reoperation on the left atrioventricular valve (both p < 0.05). CONCLUSIONS: Repair of complete atrioventricular canal using an individualized surgical approach yields reoperation and early mortality rates similar for younger infants compared with older infants, obviating the need to delay operation in symptomatic patients.
[Mh] Termos MeSH primário: Procedimentos Cirúrgicos Cardíacos/métodos
Comunicação Atrioventricular/cirurgia
Complicações Pós-Operatórias/epidemiologia
[Mh] Termos MeSH secundário: Ecocardiografia
Comunicação Atrioventricular/diagnóstico por imagem
Feminino
Seguimentos
Seres Humanos
Incidência
Lactente
Recém-Nascido
Masculino
New York/epidemiologia
Estudos Retrospectivos
Taxa de Sobrevida/tendências
Fatores de Tempo
Resultado do Tratamento
[Pt] Tipo de publicação:JOURNAL ARTICLE
[Em] Mês de entrada:1508
[Cu] Atualização por classe:161125
[Lr] Data última revisão:
161125
[Sb] Subgrupo de revista:AIM; IM
[Da] Data de entrada para processamento:150419
[St] Status:MEDLINE


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[PMID]:25870361
[Au] Autor:Lugones I; Inguanzo P; Ganum G; Di Santo M; García R
[Ad] Endereço:Fundación Favaloro University Hospital, Buenos Aires, Argentina ignaciolugones@cardiocongenitas.com.ar.
[Ti] Título:Unique combination of atrioventricular septal defect with cor triatriatum and complete vascular ring.
[So] Source:World J Pediatr Congenit Heart Surg;6(2):332-4, 2015 Apr.
[Is] ISSN:2150-136X
[Cp] País de publicação:United States
[La] Idioma:eng
[Ab] Resumo:Atrioventricular septal defect can present with one or more associated anomalies. Cor triatriatum (subdivided left atrium) and vascular rings are among the less frequent. We describe a two-month-old patient with these three cardiovascular anomalies. This case highlights the importance of exhaustive preoperative evaluation in order to achieve successful surgical correction in one stage.
[Mh] Termos MeSH primário: Coração Triatriado/diagnóstico
Comunicação Atrioventricular/diagnóstico
Defeitos dos Septos Cardíacos/diagnóstico
[Mh] Termos MeSH secundário: Anormalidades Múltiplas/diagnóstico
Anormalidades Múltiplas/diagnóstico por imagem
Anormalidades Múltiplas/cirurgia
Coração Triatriado/diagnóstico por imagem
Coração Triatriado/cirurgia
Diagnóstico Diferencial
Comunicação Atrioventricular/diagnóstico por imagem
Comunicação Atrioventricular/cirurgia
Feminino
Defeitos dos Septos Cardíacos/diagnóstico por imagem
Defeitos dos Septos Cardíacos/cirurgia
Seres Humanos
Lactente
Radiografia
[Pt] Tipo de publicação:CASE REPORTS; JOURNAL ARTICLE
[Em] Mês de entrada:1509
[Cu] Atualização por classe:161125
[Lr] Data última revisão:
161125
[Sb] Subgrupo de revista:IM
[Da] Data de entrada para processamento:150415
[St] Status:MEDLINE
[do] DOI:10.1177/2150135114564193


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[PMID]:25870336
[Au] Autor:Makhija Z; Marwah A; Mishra S; Kumar J; Goel A; Sharma R
[Ad] Endereço:Division of Congenital Cardiac Surgery, Fortis Escorts Heart Institute, New Delhi, Delhi, India zeenamakhija@gmail.com.
[Ti] Título:Biventricular repair in heterotaxy patients.
[So] Source:World J Pediatr Congenit Heart Surg;6(2):195-202, 2015 Apr.
[Is] ISSN:2150-136X
[Cp] País de publicação:United States
[La] Idioma:eng
[Ab] Resumo:BACKGROUND: Heterotaxy patients' hearts may or may not be suitable for biventricular repair depending on anatomy. Even in the subset that are amenable to surgical septation, cardiac anatomy may present multiple difficulties in achieving a satisfactory repair. However, it is also well known that heterotaxy patients are not ideal candidates for univentricular repair. METHODS: From 2007 until 2012, a total of 20 patients (11 male) with heterotaxy syndrome underwent biventricular repair (left atrial isomerism: 10 and right atrial isomerism: 10) in our center. Their median age at surgery was 40 (range: 3-108) months. Ten patients had dextrocardia. Eleven patients presented with bilateral superior vena cava, three with inferior vena cava (IVC) draining into left atrium, and six with IVC interruption with azygos or hemiazygos continuation. Anomalous pulmonary venous drainage was present in eight patients. One had a common atrium. Atrioventricular septal defect (AVSD) occurred in nine (complete AVSD in seven) patients. Eight patients had double outlet right ventricle (DORV), one had d-transposition of great arteries (d-TGA), and two had congenitally corrected transposition of the great arteries (CC-TGA). Prior palliative procedures included pulmonary artery banding in three patients and left modified Blalock-Taussig shunt in one patient. Complex intra-atrial baffle constructions were required in all patients to direct pulmonary and systemic venous inflow to the appropriate ventricle. Complete AVSDs were corrected using two-patch technique. Intraventricular tunnel repair was done for DORV. Combined atrial and arterial switch was required to rectify abnormal connections in a child with congenitally corrected transposition with normal pulmonary valve, while a Rastelli + Senning was needed in two children with CC-TGA with pulmonary atresia (n = 1) and double outlet of the right ventricle (n = 1). RESULTS: Major early postoperative complications included intestinal gangrene in four patients for which they underwent bowel resection. Two of these patients could not be salvaged. One patient required coiling of aortopulmonary collateral for early postoperative pulmonary hemorrhage. Two patients needed a tracheostomy for prolonged mechanical ventilatory support. Five patients had a pacemaker implanted for complete heart block. There were no instances of atrial baffle stenosis. Median follow-up was 27 (range: 2-46) months. There was one late death secondary to pneumonia. CONCLUSIONS: Satisfactory survival outcomes can be achieved in heterotaxy patients who undergo hemodynamically acceptable biventricular repair. Borderline ventricular hypoplasia and trivial atrioventricular valve regurgitation should not be considered as discouraging factors in anatomically suitable heterotaxy patients as it is possible to adopt a two-stage repair in such patients to achieve biventricular repair at a later stage. Anticipating a higher incidence of conduction problems and gut malrotation preemptively can help reduce the morbidity.
[Mh] Termos MeSH primário: Procedimentos Cirúrgicos Cardíacos/métodos
Síndrome de Heterotaxia/cirurgia
[Mh] Termos MeSH secundário: Bioprótese
Procedimento de Blalock-Taussig/métodos
Criança
Pré-Escolar
Dupla Via de Saída do Ventrículo Direito/cirurgia
Comunicação Atrioventricular/cirurgia
Feminino
Átrios do Coração/anormalidades
Átrios do Coração/cirurgia
Defeitos dos Septos Cardíacos
Comunicação Interatrial/cirurgia
Próteses Valvulares Cardíacas
Implante de Prótese de Valva Cardíaca/métodos
Ventrículos do Coração/cirurgia
Seres Humanos
Lactente
Masculino
Complicações Pós-Operatórias/epidemiologia
Atresia Pulmonar/cirurgia
Veias Pulmonares/anormalidades
Transposição dos Grandes Vasos/complicações
Resultado do Tratamento
[Pt] Tipo de publicação:JOURNAL ARTICLE
[Em] Mês de entrada:1510
[Cu] Atualização por classe:151119
[Lr] Data última revisão:
151119
[Sb] Subgrupo de revista:IM
[Da] Data de entrada para processamento:150415
[St] Status:MEDLINE
[do] DOI:10.1177/2150135114563772



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