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Introdução: A Síndrome do Coração Esquerdo Hipoplásico (SCEH) compreende um espectro de malformações estruturais cardíacas caracterizadas por um hipodesenvolvimento significativo do complexo coração esquerdo-aorta, que apesar da evolução do tratamento, continua sendo um desafio. O objetivo deste trabalho é identificar diferenças morfológicas e histológicas em corações com atresia e estenose mitral na SCEH. Métodos: Estudo de 33 corações com SCEH e nove corações normais (controle), divididos em dois grupos, atresia mitral (AM) e estenose mitral (EM), obtidos em necrópsia e submetidos a análise morfológica dos segmentos da aorta, características da valva mitral e tricúspide, septo inter atrial, miocárdio, cavidades ventriculares e análise histológica com as colorações e hematoxilina/eosina e picro-sírius. Resultados: Observamos nove espécimes com Atresia Mitral e Atresia Aórtica (AMAA), 27,2%; treze com Atresia Mitral e Estenose Aórtica (AMEA), 39,3% e onze com Estenose Mitral e Estenose Aórtica (EMEA) 33,3%. Encontramos associação significativa de predominância de coronárias tortuosas no grupo EM (?2= 4,911; P=0,027) e a dominância coronariana esquerda está em 75% dos casos de EM, com diferença significativa entre os dois grupos (?2=9,298; P=0,01). No grupo AM encontramos correlação significativa entre aorta descendente e arco aórtico (r =0,692; P=0,039) e entre aorta descendente e istmo aórtico (r=0,796; P=0,010).No grupo EM, há correlação significativa entre as variáveis: Anel mitral e comprimento via de entrada de ventrículo direito (r=0,523; P=0,045); Anel mitral e istmo aórtico (r=0,692; P=0,003); ventrículo esquerdo cavidade e aorta ascendente (r=0,643; P=0,010); Arco aórtico e istmo aórtico (r=0,678; P=0,001); Aorta ascendente e arco aórtico (r=0,444; P = 0,044). Não existe diferença significativa no tamanho dos miócitos (coloração HE) entre o grupo AMAA e o grupo EMAA/EMEA (P=0,427), porém existe diferença significativa entre AMAA e controle (P=0,011) e entre EMAA/EMEA e controle (P=0,023). O percentual de colágeno (coloração de picro-sírius) é significantemente diferente entre os três grupos (P=0,0001) e o grupo AM é o que contém maior percentual de colágeno. Conclusões: 1. Na SCEH os corações com EM apresentam significativamente coronárias tortuosas e dominância coronariana esquerda em comparação com AM; 2. No grupo AM encontramos correlação significativa entre o diâmetro da aorta descendente e arco aórtico e entre aorta descendente e istmo aórtico; 3. No grupo EM, há correlação significativa entre as seguintes variáveis: anel mitral e comprimento via de entrada do ventrículo direito, anel mitral e istmo aórtico, cavidade do ventrículo esquerdo e aorta ascendente, arco aórtico e istmo aórtico e arco aórtico e aorta ascendente; 4. Há hipertrofia dos miócitos nos espécimes com AM e EM em comparação com o grupo controle; 5. Na SCEH o percentual de colágeno é superior ao grupo controle; 6. O grupo AM tem maior percentual de colágeno que o grupo EM / Introduction: Hypoplastic left heart syndrome (HLHS) comprises a spectrum of cardiac malformations characterized by a significant underdevelopment of the left heart-aorta complex, which remains a challenge despite the progress of treatment. The objective of this work is to identify morphological and histological differences in hearts with atresia and mitral stenosis with HLHS. Methods: 33 hearts with HLHS divided into two groups, mitral atresia (AM) and mitral stenosis (MS) and nine normal hearts (control),obtained at autopsy, submitted to morphological analysis of aortic segments, mitral and tricuspid valves, atrial septum, infarction, and ventricular cavities and histological study with Hematoxylin/eosin and picro sírius stain. Results: There were nine specimens with aortic atresia and mitral atresia (AMAA), 27.2%; thirteen with atresia Mitral and Aortic Stenosis (AMEA), 39.3% and eleven with Mitral Stenosis and Aortic Stenosis (EMEA) 33.3%. There is a significant association of prevalence of coronary tortuous in the MS group (x2 = 4.911, P=0.027) and left coronary dominance in 75% of cases of MS, with a significant difference between the two groups (x2 2 = 9.298, P=0,01). In the AM group we found a significant correlation between the descending aorta and aortic arch (r=0.692, P=0.039) and between the descending aorta and aortic isthmus (r =0.796, P=0.01). In the MS group there was a significant correlation between variables: mitral ring and length inlet right ventricle (r= 0.523, P=0.045), mitral and aortic isthmus (r=0.692, P=0.003), left ventricular cavity and ascending aorta (r=0.643, P=0.01); Aortic arch and aortic isthmus (r=0.678, P=0.001), ascending aorta and aortic arch (r=0.444, P=0.044).There is no significant difference in the size of myocites (HE staining) between the group and the group AMAA EMAA / EMEA (P = 0.427), but we found significant difference between AMAA and control (P = 0.011) and between EMAA / EMEA and control (P=0.023). The percentage of collagen (picrosirius staining) is different between the three groups (P=0.0001) and AM is the group that contains a higher percentage of collagen. Conclusions: 1. In HLHS hearts with MS present significant tortuous coronary and left coronary dominance compared with AM; 2. In the AM group there is a significant correlation between the diameter of the descending aorta and aortic arch and descending aorta and across the aortic isthmus; 3. In the MS group, there was significant correlation between the following variables: length and mitral inflow tract, mitral and aortic isthmus, left ventricular cavity and the ascending aorta, aortic arch and isthmus aorta and aortic arch and ascending aorta; 4. There is myocite hypertrophy in specimens with AM and EM compared with control; 5. In the HSLS collagen percentage is higher than control; 6. The AM group has a higher percentage of collagen than the EM group

Introdução: A Síndrome do Coração Esquerdo Hipoplásico (SCEH) compreende um espectro de malformações estruturais cardíacas caracterizadas por um hipodesenvolvimento significativo do complexo coração esquerdo-aorta, que apesar da evolução do tratamento, continua sendo um desafio. O objetivo deste trabalho é identificar diferenças morfológicas e histológicas em corações com atresia e estenose mitral na SCEH. Métodos: Estudo de 33 corações com SCEH e nove corações normais (controle), divididos em dois grupos, atresia mitral (AM) e estenose mitral (EM), obtidos em necrópsia e submetidos a análise morfológica dos segmentos da aorta, características da valva mitral e tricúspide, septo inter atrial, miocárdio, cavidades ventriculares e análise histológica com as colorações e hematoxilina/eosina e picro-sírius. Resultados: Observamos nove espécimes com Atresia Mitral e Atresia Aórtica (AMAA), 27,2%; treze com Atresia Mitral e Estenose Aórtica (AMEA), 39,3% e onze com Estenose Mitral e Estenose Aórtica (EMEA) 33,3%. Encontramos associação significativa de predominância de coronárias tortuosas no grupo EM (?2= 4,911; P=0,027) e a dominância coronariana esquerda está em 75% dos casos de EM, com diferença significativa entre os dois grupos (?2=9,298; P=0,01). No grupo AM encontramos correlação significativa entre aorta descendente e arco aórtico (r =0,692; P=0,039) e entre aorta descendente e istmo aórtico (r=0,796; P=0,010).No grupo EM, há correlação significativa entre as variáveis: Anel mitral e comprimento via de entrada de ventrículo direito (r=0,523; P=0,045); Anel mitral e istmo aórtico (r=0,692; P=0,003); ventrículo esquerdo cavidade e aorta ascendente (r=0,643; P=0,010); Arco aórtico e istmo aórtico (r=0,678; P=0,001); Aorta ascendente e arco aórtico (r=0,444; P = 0,044). Não existe diferença significativa no tamanho dos miócitos (coloração HE) entre o grupo AMAA e o grupo EMAA/EMEA (P=0,427), porém existe diferença significativa entre AMAA e controle (P=0,011) e entre EMAA/EMEA e controle (P=0,023). O percentual de colágeno (coloração de picro-sírius) é significantemente diferente entre os três grupos (P=0,0001) e o grupo AM é o que contém maior percentual de colágeno. Conclusões: 1. Na SCEH os corações com EM apresentam significativamente coronárias tortuosas e dominância coronariana esquerda em comparação com AM; 2. No grupo AM encontramos correlação significativa entre o diâmetro da aorta descendente e arco aórtico e entre aorta descendente e istmo aórtico; 3. No grupo EM, há correlação significativa entre as seguintes variáveis: anel mitral e comprimento via de entrada do ventrículo direito, anel mitral e istmo aórtico, cavidade do ventrículo esquerdo e aorta ascendente, arco aórtico e istmo aórtico e arco aórtico e aorta ascendente; 4. Há hipertrofia dos miócitos nos espécimes com AM e EM em comparação com o grupo controle; 5. Na SCEH o percentual de colágeno é superior ao grupo controle; 6. O grupo AM tem maior percentual de colágeno que o grupo EM / Introduction: Hypoplastic left heart syndrome (HLHS) comprises a spectrum of cardiac malformations characterized by a significant underdevelopment of the left heart-aorta complex, which remains a challenge despite the progress of treatment. The objective of this work is to identify morphological and histological differences in hearts with atresia and mitral stenosis with HLHS. Methods: 33 hearts with HLHS divided into two groups, mitral atresia (AM) and mitral stenosis (MS) and nine normal hearts (control),obtained at autopsy, submitted to morphological analysis of aortic segments, mitral and tricuspid valves, atrial septum, infarction, and ventricular cavities and histological study with Hematoxylin/eosin and picro sírius stain. Results: There were nine specimens with aortic atresia and mitral atresia (AMAA), 27.2%; thirteen with atresia Mitral and Aortic Stenosis (AMEA), 39.3% and eleven with Mitral Stenosis and Aortic Stenosis (EMEA) 33.3%. There is a significant association of prevalence of coronary tortuous in the MS group (x2 = 4.911, P=0.027) and left coronary dominance in 75% of cases of MS, with a significant difference between the two groups (x2 2 = 9.298, P=0,01). In the AM group we found a significant correlation between the descending aorta and aortic arch (r=0.692, P=0.039) and between the descending aorta and aortic isthmus (r =0.796, P=0.01). In the MS group there was a significant correlation between variables: mitral ring and length inlet right ventricle (r= 0.523, P=0.045), mitral and aortic isthmus (r=0.692, P=0.003), left ventricular cavity and ascending aorta (r=0.643, P=0.01); Aortic arch and aortic isthmus (r=0.678, P=0.001), ascending aorta and aortic arch (r=0.444, P=0.044).There is no significant difference in the size of myocites (HE staining) between the group and the group AMAA EMAA / EMEA (P = 0.427), but we found significant difference between AMAA and control (P = 0.011) and between EMAA / EMEA and control (P=0.023). The percentage of collagen (picrosirius staining) is different between the three groups (P=0.0001) and AM is the group that contains a higher percentage of collagen. Conclusions: 1. In HLHS hearts with MS present significant tortuous coronary and left coronary dominance compared with AM; 2. In the AM group there is a significant correlation between the diameter of the descending aorta and aortic arch and descending aorta and across the aortic isthmus; 3. In the MS group, there was significant correlation between the following variables: length and mitral inflow tract, mitral and aortic isthmus, left ventricular cavity and the ascending aorta, aortic arch and isthmus aorta and aortic arch and ascending aorta; 4. There is myocite hypertrophy in specimens with AM and EM compared with control; 5. In the HSLS collagen percentage is higher than control; 6. The AM group has a higher percentage of collagen than the EM group

BACKGROUND: Hypoplastic Left Heart Syndrome is a congenital birth defect that is defined by underdevelopment of the left heart during pregnancy. This is especially dangerous as the left heart holds the systemic flow of blood- the oxygenated blood. Not enough oxygen throughout the whole body causes cyanosis, which symptoms include bluish discoloration of the skin or mucous membrane due to low oxygen saturation. Single Ventricle Palliation followed by Biventricular Conversion is the most common surgical procedural pathway to correct this defect. The goal is to convert from a single ventricle circulation during single ventricle palliation to biventricular circulation via biventricular conversion, which is the normal heart anatomy. Single Ventricle Pallation consists of three stages: Stage 1 Norwood Procedure, Bidirectional Glenn, and Fontan. Biventricular Conversion can be performed after any of the three stages. In addition, further compromise of the left ventricle includes other factors such as a thickening of fibroblast-like cells on the endocardial layer called endocardial fibroelastosis. Therefore, additional surgical procedures, also known as recruitment procedures, combat these problems. It is critical to find a correlation between a specific procedure and post surgery success in left ventricle growth and function for these patients. OBJECTIVES: Patients with Hypoplastic Left Heart Syndrome at Boston Children’s Hospital have undergone single ventricle palliation with some patients proceeding to biventricular conversion. This study aimed to study the palliation stages individually and recruitment procedures (specifically endocardial fibroelastosis resection) on the effect of left ventricle growth. METHODS: Patients with Hypoplastic Left Heart Syndrome were studied retrospectively (before 2014) and prospectively (after 2014 until December 1, 2018). Single Ventricle Palliation and Biventricular Conversion were analyzed via descriptional analysis with evidence of left ventricular growth measured by left ventricular end diastolic volume and respective z-scores. Z-scores were used to standardize end diastolic volume values across variability in age, weight, and height. RESULTS: A total of 55 patients underwent single ventricle palliation and 39 ended with biventricular circulation via biventricular conversion. Overall, there was a 9.29 ml increase in end diastolic volume between Bidirectional Glenn and Fontan and a 0.795 increase in end diastolic volume z-score between Fontan and Biventricular Conversion. Next, those who did not have recruitment procedures experienced a 135.6%, 48.8%, and 0% growth at Stage 1, Bidirectional Glenn, and Fontan, respectively, before directly proceeding to biventricular conversion. Those with recruitment experienced a 44.5%, 90.4%, and 83.0% growth at Stage 1, Bidirectional Glenn, and Fontan, respectively, before directly proceeding to biventricular conversion. Finally, there was a 50.2% and 62.3% in left ventricular growth at Bidirectional Glenn and Fontan, respectively, after endocardial fibroelastosis resection compared to only a 6.9% growth at Stage 1. CONCLUSION: Bidirectional Glenn was the most effective palliation stage for left ventricular growth. Recruitment in patients at this stage was associated with growth that exceeds those who did not have recruitment. This stage also best demonstrates the ability and success of growing a small ventricle to be adequate for biventricular conversion. Left ventricular growth at Fontan circulation holds promising results that are a point of interest for more studies. Endocardial Fibroelastosis resection is more effective on left ventricular growth at Bidirectional Glenn and Fontan compared to Stage 1.