Case Report: The Challenge for Diagnosis of Myocarditis and Transplant Rejection After COVID Infection in a Heart-Transplanted Adolescent.

Patients who have undergone organ transplantation are immunosuppressed hosts, leaving them at a higher risk of infections. SARS-COV-2 has been shown to affect heart-transplanted patients. In this case report, we present the case of a 14-year-old heart transplant recipient who developed signs and symptoms of heart failure, along with fatigue, after a COVID-19 infection. An endomyocardial biopsy was performed to diagnose rejection and to evaluate whether this was myocarditis due to SARS-COV-2. The biopsy showed intense acute cellular rejection (3R) and antibody rejection PAMR1 H+ but was negative for the SARS-CoV-2 virus. The patient received organ rejection therapy with high-dose methylprednisolone and human immunoglobulin. After treatment, her heart function recovered, with biopsy investigations showing a lower level of cellular rejection (1R).

The impact of preexisting myocardial remodeling on ventricular function early after tetralogy of Fallot repair.

BACKGROUND: Twenty-three patients (median age 23 months) who underwent Fallot's tetralogy repair were investigated prospectively to detect a possible association between histopathologic myocardial remodeling and echocardiographic findings of systolic or diastolic ventricular dysfunction. METHODS: Intraoperatively resected infundibular bands and subendocardial biopsy samples from the right ventricle (RV) and left ventricle were obtained for histopathologic evaluation. Tissue Doppler echocardiographic interrogation of the ventricles was performed before surgery and in the postoperative period. RESULTS: Histopathologic data revealed hypertrophy of the RV cardiomyocytes and increased interstitial collagen in both ventricles. Mean values of RV isovolumic acceleration decreased significantly at the third evaluation compared with the preoperative values (P = .006). RV myocardial fibrosis greater than 8.3% was associated with a probability of altered E' of at least 0.7 (odds ratio = 2.31). CONCLUSION: Preoperative histologic myocardial remodeling influenced the postoperative RV function in this group of patients with late repair. Further studies are necessary to evaluate the myocardium in younger patients and to define its influence in the long-term follow-up.

Seguimento de dois anos em pacientes com hipertensão arterial pulmonar sob tratamento com sildenafila / Two-year follow-up of pulmonary arterial hypertension patients treated with sildenafil

FUNDAMENTO: Os efeitos de longo prazo das drogas desenvolvidas para o controle da hipertensão arterial pulmonar (HAP) são pouco conhecidos, já que os estudos multicêntricos em geral têm duração de 12 a 16 semanas. OBJETIVO: Avaliar a evolução de dois anos, em pacientes com HAP submetidos à monoterapia com sildenafila (inibidor da fosfodiesterase-5), com respeito à capacidade funcional. MÉTODOS: Vinte e quatro pacientes (idade entre 8 e 54 anos) com HAP idiopática (HAPI, n = 9) ou associada a cardiopatias congênitas (HAP-CCg, n = 15) foram tratados com sildenafila por dois anos, com doses diárias que variaram de 60 a 225 mg (três tomadas), por via oral. A capacidade física foi avaliada pela distância caminhada no teste de 6 minutos (DC6M) e pelo grau de dispneia ao final da caminhada (escala de Borg), sendo também registrada a saturação periférica de oxigênio (SpO(2)6M, oximetria de pulso). RESULTADOS: Nos 18 pacientes que completaram dois anos de seguimento, houve incremento progressivo e sustentado na DC6M, tanto no grupo HAPI (de 239 ± 160 m para 471 ± 66 m, p = 0,0076) como no grupo HAP-CCg (de 361 ± 144 m para 445 ± 96m, p = 0,0031), com melhora da dispneia ao final da caminhada (p < 0,05 em ambos). Não houve decréscimo na SpO(2)6M nos grupos considerados; em particular, pacientes com HAP-CCg evoluíram de 77 ± 20 por cento para 79 ± 16 por cento (p = 0,5248). Houve 5 óbitos (três no grupo HAPI) e uma perda de seguimento no período. CONCLUSÃO: Em dois anos de seguimento, a sildenafila mostrou-se útil no controle da condição funcional de pacientes com HAP, com melhora significante nas duas etiologias consideradas.

BACKGROUND: The long-term effects of drugs developed for the control of pulmonary arterial hypertension (PAH) are little known, since multicenter studies usually last 12 to 16 weeks. OBJECTIVE: To evaluate the two-year outcome of PAH patients receiving monotherapy with sildenafil (a phosphodiesterase-5 inhibitor), with regard to their functional capacity. METHODS: Twenty four patients (ages between 8 and 54 years) with idiopathic PAH (IPAH, n = 9) or congenital heart disease-associated PAH (CHD-PAH, n = 15) were treated with sildenafil for two years, with daily oral doses ranging from 60 to 225 mg (tid). Physical capacity was assessed by the distance walked in the 6-minute walk test (DW6M) and by the degree of dyspnea at the end of the walk (Borg scale); peripheral oxygen saturation was also recorded (SpO(2)6M, pulse oximetry). RESULTS: In the 18 patients who completed the two-year follow-up, there was a progressive and sustained increase in DW6M, both in the IPAH group (from 239 ± 160 m to 471 ± 66 m, p = 0.0076) and in the CHD-PAH group (from 361 ± 144 m to 445 ± 96m, p = 0.0031), with improvement of dyspnea at the end of the walk (p<0.05 for both groups). No decrease in SpO(2)6M was observed in the groups; in patients with CHD-PAH, in particular, SpO(2)6M went from 77 ± 20 percent to 79 ± 16 percent (p = 0.5248). Five deaths occurred (three in the IPAH group) and one patient was lost to follow-up during the study period. CONCLUSION: In a two-year follow-up, sildenafil proved useful in the control of the functional status of PAH patients, with significant improvement in both groups considered.

[Two-year follow-up of pulmonary arterial hypertension patients treated with sildenafil]. / Seguimento de dois anos em pacientes com hipertensão arterial pulmonar sob tratamento com sildenafila.

BACKGROUND: The long-term effects of drugs developed for the control of pulmonary arterial hypertension (PAH) are little known, since multicenter studies usually last 12 to 16 weeks. OBJECTIVE: To evaluate the two-year outcome of PAH patients receiving monotherapy with sildenafil (a phosphodiesterase-5 inhibitor), with regard to their functional capacity. METHODS: Twenty four patients (ages between 8 and 54 years) with idiopathic PAH (IPAH, n = 9) or congenital heart disease-associated PAH (CHD-PAH, n = 15) were treated with sildenafil for two years, with daily oral doses ranging from 60 to 225 mg (tid). Physical capacity was assessed by the distance walked in the 6-minute walk test (DW6M) and by the degree of dyspnea at the end of the walk (Borg scale); peripheral oxygen saturation was also recorded (SpO(2)6M, pulse oximetry). RESULTS: In the 18 patients who completed the two-year follow-up, there was a progressive and sustained increase in DW6M, both in the IPAH group (from 239 +/- 160 m to 471 +/- 66 m, p = 0.0076) and in the CHD-PAH group (from 361 +/- 144 m to 445 +/- 96 m, p = 0.0031), with improvement of dyspnea at the end of the walk (p<0.05 for both groups). No decrease in SpO(2)6M was observed in the groups; in patients with CHD-PAH, in particular, SpO(2)6M went from 77 +/- 20% to 79 +/- 16% (p = 0.5248). Five deaths occurred (three in the IPAH group) and one patient was lost to follow-up during the study period. CONCLUSION: In a two-year follow-up, sildenafil proved useful in the control of the functional status of PAH patients, with significant improvement in both groups considered.

The myocardium in tetralogy of Fallot: a histological and morphometric study.

BACKGROUND: Patients with Tetralogy of Fallot frequently develop ventricular dysfunction in the postoperative period. The histological basis of this functional alteration has been scarcely studied. OBJECTIVE: To evaluate myocardial remodeling in anatomical specimens, comparing the subepicardial and subendocardial regions, especially because the subendocardial region is easily approached by means of endomyocardial biopsy. METHODS: Transmural sections of myocardium from the right ventricular (RV) inflow tract, anterior wall and infundibulum, and from the left ventricular (LV) free wall were evaluated regarding the degree of cardiomyocyte hypertrophy, vascularization and interstitial fibrosis were analyzed. RESULTS: The mean diameter of subendocardial cardiomyocytes is similar to that of subepicardial cardiomyocytes in all regions, except for the RV infundibulum, in which subendocardial cardiomyocytes are significantly larger in relation to those of the subepicardium (p=0.007). The amount of interstitial collagen is in the upper limits of normal and was similar in the subendocardial layers in comparison with the subpericardial layer of each region; however, it was greater in the inflow tract and RV anterior wall than in the LV lateral wall. The numerical density of subendocardial capillaries was similar to that of the subepicardium and was lower than the mean minus two standard deviations of normal in all regions and layers, except for the infundibulum, in which the subepicardium showed normal values and the subendocardium showed values lower than the mean minus two standard deviations. CONCLUSION: The postnatal myocardial changes in Tetralogy of Fallot are homogeneously distributed in the subepicardial and subendocardial halves of the ventricular walls, except for the infundibulum, which has peculiar remodeling characteristics and, therefore, is not representative of the other ventricular regions and layers for morphometric studies.

O miocárdio na tetralogia de Fallot: estudo histológico e morfométrico / The myocardium in tetralogy of Fallot: a histological and morphometric study / El miocardio en la tetralogía de Fallot: estudio histológico y morfométrico

FUNDAMENTO: Pacientes com tetralogia de Fallot freqüentemente cursam com disfunção ventricular no período pós-operatório. A base histológica dessa alteração funcional tem sido pouco estudada. OBJETIVO: Avaliar, em espécimes anatômicos, o remodelamento miocárdico comparando as regiões subepicárdica e subendocárdica, especialmente por esta última ser facilmente abordável por meio de biópsias endomiocárdicas. MÉTODOS:Análises em cortes transmurais de miocárdio da via de entrada, parede anterior e infundíbulo do ventrículo direito (VD) e da parede livre do esquerdo (VE), foram avaliados quanto ao grau de hipertrofia de cardiomiócitos, de vascularização e fibrose intersticial. RESULTADOS:O diâmetro médio dos cardiomiócitos do subendocárdio é semelhante ao do subepicárdio em todas as regiões, com exceção do infundíbulo do VD, em que os subendocárdicos se mostraram significativamente maiores em relação aos do subepicárdio (p=0,007). A quantidade de colágeno intersticial encontra-se nos limites superiores do normal e foi similar nas camadas subendocárdicas, comparada à subpericárdica de cada região, sendo, todavia, maior na via de entrada e na parede anterior do VD, do que na parede lateral do VE. A densidade numérica de capilares do subendocárdio foi semelhante à do subepicárdio e esteve menor que a média menos dois desvios-padrão do normal em todas as regiões e camadas, com exceção do infundíbulo, em que o subepicárdio mostrava valores normais e o subendocárdio valores menores que a média menos dois desvios-padrão. CONCLUSÃO: As alterações do miocárdio pós-natal na tetralogia de Fallot estão distribuídas homogeneamente nas metades subepicárdica e subendocárdica das paredes ventriculares, com exceção do infundíbulo, que apresenta características peculiares de remodelamento e que, portanto, não é representativo das demais regiões e camadas ventriculares para estudos morfométricos.

BACKGROUND: Patients with Tetralogy of Fallot frequently develop ventricular dysfunction in the postoperative period. The histological basis of this functional alteration has been scarcely studied. OBJECTIVE: To evaluate myocardial remodeling in anatomical specimens, comparing the subepicardial and subendocardial regions, especially because the subendocardial region is easily approached by means of endomyocardial biopsy. METHODS: Transmural sections of myocardium from the right ventricular (RV) inflow tract, anterior wall and infundibulum, and from the left ventricular (LV) free wall were evaluated regarding the degree of cardiomyocyte hypertrophy, vascularization and interstitial fibrosis were analyzed. RESULTS: The mean diameter of subendocardial cardiomyocytes is similar to that of subepicardial cardiomyocytes in all regions, except for the RV infundibulum, in which subendocardial cardiomyocytes are significantly larger in relation to those of the subepicardium (p=0.007). The amount of interstitial collagen is in the upper limits of normal and was similar in the subendocardial layers in comparison with the subpericardial layer of each region; however, it was greater in the inflow tract and RV anterior wall than in the LV lateral wall. The numerical density of subendocardial capillaries was similar to that of the subepicardium and was lower than the mean minus two standard deviations of normal in all regions and layers, except for the infundibulum, in which the subepicardium showed normal values and the subendocardium showed values lower than the mean minus two standard deviations. CONCLUSION: The postnatal myocardial changes in Tetralogy of Fallot are homogeneously distributed in the subepicardial and subendocardial halves of the ventricular walls, except for the infundibulum, which has peculiar remodeling characteristics and, therefore, is not representative of the other ventricular regions and layers for morphometric studies.

FUNDAMENTO: Frecuentemente, pacientes con tetralogía de Fallot cursan con disfunción ventricular en el período postoperatorio. La base histológica de esa alteración funcional ha sido poco estudiada. OBJETIVO: Evaluar, en especímenes anatómicos, la remodelación miocárdica comparándose las regiones subepicárdica y subendocárdica, sobre todo por esta última ser fácilmente abordable por medio de biopsias endomiocárdicas. MÉTODOS: Se evaluaron análisis en cortes transmurales de miocardio de la vía de entrada, pared anterior e infundíbulo del ventrículo derecho (VD) y de la pared libre del izquierdo (VI), en cuanto al grado de hipertrofia de cardiomiocitos, de vascularización y fibrosis intersticial. RESULTADOS: El diámetro promedio de los cardiomiocitos del subendocardio se asemeja al del subepicardio en todas las regiones, con excepción del infundíbulo del VD, en que los subendocárdicos se mostraron significativamente mayores con relación a los del subepicardio (p=0,007). La cantidad de colágeno intersticial se encuentra en los límites superiores al normal y fue similar en las camadas subendocárdicas, comparada a la subpericárdica de cada región; pero se mostró mayor en la vía de entrada y en la pared anterior del VD, que en la pared lateral del VI. La densidad numérica de capilares del subendocardio se asemejó a la del subepicardio y se mostró menor que el promedio menos dos desviaciones estándar del normal, en todas las regiones y capas, con excepción del infundíbulo, en lo que el subepicardio revelaba valores normales y el subendocardio valores menores que el promedio menos dos desviaciones estándar. CONCLUSIÓN: Las alteraciones del miocardio postnatal en la tetralogía de Fallot están distribuidas de modo homogéneo en las mitades subepicárdica y subendocárdica de las paredes ventriculares, a excepción del infundíbulo, que presenta características peculiares de remodelación y que, por lo tanto, no es representativo de las otras regiones...