RESUMEN
OBJECTIVES: In children, it is difficult to control mitral valve (MV) or tricuspid valve (TV) regurgitation with conventional procedures alone because complex factors hamper easy improvement of valve coaptation. We investigated interannular bridging in children with MV/TV regurgitation. METHODS: The subjects were 9 patients who underwent interannular bridging to control TV or MV regurgitation between January 2014 and December 2021. We analysed reintervention for the valve, progression of stenosis/regurgitation and valve growth. RESULTS: The TV and MV groups included 4 and 5 patients, respectively. At operation, the median age was 5.8 (1.4-14) years in TV and 3.6 (0.3-7.0) years in MV. The median weight was 13.0 (8.4-41.2) kg in TV and 11.0 (4.8-18.3) kg in MV. The median follow-up periods were 78 (11-94) months for TV and 30 (4-34) months for MV. None of the patients in either group underwent reintervention. Moderate or greater regurgitation recurred in 1 TV patient but subsequently improved to mild regurgitation. Valve stenosis (mean diastolic pressure gradient >10 mmHg) was not detected. The median valve diameter (Z-score) ranged from -1.17 (-3.7 to 0.85) at discharge to -0.59 (-1.2 to 2.01) at the latest follow-up in TV patients. In MV patients, valve diameter changed from 1.14 (-1.68 to 1.46) to 0.72 (-0.23 to 1.36). After bridging, the coaptation height was maintained at the same value over time. CONCLUSIONS: Interannular bridging could be a useful approach for complicated TV/MV regurgitation in children.
RESUMEN
Intrapulmonary-artery septoplasty may be effective for establishing two-lung Fontan circulation in patients with unilateral pulmonary circulation. However, evaluation of the function of each lung by conventional modalities can be challenging in these patients due to differing sources of blood flow to the left and right lungs following intrapulmonary-artery septation. Herein, we report a case in which two-lung Fontan circulation was successfully achieved after using cardiac MRI along with conventional modalities to evaluate pulmonary circulation.
Asunto(s)
Procedimiento de Fontan , Cardiopatías Congénitas , Humanos , Arteria Pulmonar/diagnóstico por imagen , Arteria Pulmonar/cirugía , Imagen por Resonancia Magnética , Circulación Pulmonar/fisiología , Hemodinámica , Cardiopatías Congénitas/diagnóstico por imagen , Cardiopatías Congénitas/cirugíaRESUMEN
The primum atrial septal defect suture line adjacent to the crux is performed carefully with various techniques to avoid atrioventricular block in repair of complete atrioventricular septal defect. We describe our technical modification to simplify the shallow suture line only into the endocardium above the atrioventricular node without conduction disturbance.
Asunto(s)
Defectos del Tabique Interatrial , Defectos del Tabique Interventricular , Defectos de los Tabiques Cardíacos , Humanos , Nodo Atrioventricular , Defectos del Tabique Interventricular/cirugía , Técnicas de Cierre de Heridas , Suturas , Defectos del Tabique Interatrial/cirugíaRESUMEN
OBJECTIVES: Cardiac malformations are a major component of heterotaxy syndrome that results in significant mortality and morbidity, especially in patients with a single ventricle and right isomerism (RI). The goal of this study was to evaluate the mortality after cardiovascular surgery in patients with a functional single ventricle and RI over a long follow-up period (â¼40 years) and to determine the predicted risk factors for mortality. METHODS: We performed a retrospective review of the medical records of 129 consecutive patients with functional single ventricle and RI who underwent pulmonary flow control operations at Mt. Fuji Shizuoka Children's Hospital between 1979 and 2020. To evaluate mortality rates, the patients were divided into 2 groups (era 1: 1979-1999 and era 2: 2000-2020) based on the date of the first-stage palliation. RESULTS: The estimated survival rate at 10 years was 36.4% in era 1 and 57.8% in era 2. The estimated survival rate improved significantly (P = 0.0268) between the 2 eras. The rate of Fontan procedure completion was also significantly better in the current era (P = 0.0392; 22/59 in era 1 and 38/66 in era 2). In the multivariable analysis, the date of the first-stage palliation was the only predictor of mortality. CONCLUSIONS: The mortality rate after cardiovascular surgery in patients with a functional single ventricle and RI has improved over the past 20 years; however, it still remains high. This improvement may be attributed to our current surgical strategy and clinical management; however, further investigations are needed to prove this observation.
Asunto(s)
Procedimiento de Fontan , Cardiopatías Congénitas , Síndrome de Heterotaxia , Corazón Univentricular , Niño , Cardiopatías Congénitas/cirugía , Ventrículos Cardíacos/anomalías , Ventrículos Cardíacos/cirugía , Síndrome de Heterotaxia/cirugía , Humanos , Lactante , Isomerismo , Estudios Retrospectivos , Factores de Riesgo , Resultado del TratamientoRESUMEN
A boy diagnosed with persistent truncus arteriosus and severe truncal valve regurgitation had uncontrollable heart failure. Thus, truncal valve repair, ventricular septal defect closure, and right ventricular outflow tract reconstruction were performed on the second day of life. We report about a neonate with severe truncal valve regurgitation who was successfully treated with autologous pericardial truncal valve leaflet reconstruction.
Asunto(s)
Cardiopatías Congénitas , Enfermedades de las Válvulas Cardíacas , Tronco Arterial Persistente , Cardiopatías Congénitas/cirugía , Enfermedades de las Válvulas Cardíacas/cirugía , Válvulas Cardíacas , Humanos , Recién Nacido , Masculino , Pericardio/cirugía , Tronco Arterial Persistente/cirugíaRESUMEN
OBJECTIVE: Patients with unbalanced pulmonary artery growth resulting from pulmonary coarctation are considered unsuitable candidates for the Fontan procedure. Particularly, patients with right isomerism pose a challenge. We aimed to investigate the use of primary central pulmonary artery plasty at initial palliation in patients with right isomerism. METHODS: We recruited 34 right isomerism patients with pulmonary atresia and pulmonary coarctation who underwent modified Blalock-Taussig shunt with or without primary central pulmonary artery plasty between 1998 and 2014. We classified them into the primary central pulmonary artery plasty (group P) and no primary central pulmonary artery plasty (group N) groups. We retrospectively analyzed reintervention for pulmonary artery after initial palliation, difference in size between the left and right pulmonary arteries, overall survival, success of the Fontan procedure. RESULTS: The group P and group N included 25 and 9 patients, respectively. Five (20%) and six (67%) patients in group P and group N, respectively, required reintervention for pulmonary artery after initial palliation (p = 0.017). No patient underwent reintervention for the pulmonary artery before bidirectional cavopulmonary shunt in group P. There was a significant difference in the bilateral pulmonary artery size balance between the groups before bidirectional cavopulmonary shunt (p = 0.041). The two-lung Fontan procedure was successful in 14 (56%) and 1 (11%) patient in group P and group N. CONCLUSION: Primary central pulmonary artery plasty may contribute toward improving the balance in the size of the PA and preclude the need for reintervention for PA.
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Coartación Aórtica , Procedimiento de Fontan , Síndrome de Heterotaxia , Coartación Aórtica/diagnóstico por imagen , Coartación Aórtica/cirugía , Procedimiento de Fontan/efectos adversos , Procedimiento de Fontan/métodos , Síndrome de Heterotaxia/cirugía , Humanos , Lactante , Pulmón/cirugía , Arteria Pulmonar/diagnóstico por imagen , Arteria Pulmonar/cirugía , Estudios Retrospectivos , Resultado del TratamientoRESUMEN
A boy was diagnosed with a unicuspid aortic valve with severe stenosis at birth. Percutaneous balloon aortic valvuloplasty was performed four times; however, he had severe heart and growth failure. Thus, aortic valve repair was performed at age 2 years and 6 months. He weighed 6.6 kg. The aortic valve annulus diameter was 9.8 mm (z value = 0.43). We report on a small toddler with congenital aortic stenosis who was successfully treated with autologous pericardial aortic valve leaflet reconstruction using the open-sleeve technique.
Asunto(s)
Insuficiencia de la Válvula Aórtica , Estenosis de la Válvula Aórtica , Procedimientos Quirúrgicos Cardíacos , Enfermedades de las Válvulas Cardíacas , Válvula Aórtica/anomalías , Válvula Aórtica/diagnóstico por imagen , Válvula Aórtica/cirugía , Insuficiencia de la Válvula Aórtica/diagnóstico por imagen , Insuficiencia de la Válvula Aórtica/etiología , Insuficiencia de la Válvula Aórtica/cirugía , Estenosis de la Válvula Aórtica/diagnóstico por imagen , Estenosis de la Válvula Aórtica/cirugía , Preescolar , Enfermedades de las Válvulas Cardíacas/cirugía , Humanos , Recién Nacido , Masculino , Resultado del TratamientoRESUMEN
Staphylococcus lugdunensis infective endocarditis (IE) is very rare in children. A female neonote presented with fever on the 29th postoperative day after undergoing a modified Norwood procedure (right ventricular-pulmonary artery [RV-PA] conduit). Blood cultures were positive for S. lugdunensis. Echocardiography did not demonstrate vegetation. Therefore, we made a diagnosis of catheter-related bacteremia instead of IE. Cultures were negative 3 days after treatment initiation with intravenous vancomycin. One month after discharge, she developed acute hypoxia and could not be revived because of obstruction of RV-PA conduit with large vegetation. Even in S. lugdunensis bacteremia without IE, surgical treatment should be considered early.
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Bacteriemia , Embolia , Endocarditis Bacteriana , Infecciones Estafilocócicas , Staphylococcus lugdunensis , Antibacterianos/uso terapéutico , Bacteriemia/diagnóstico , Bacteriemia/tratamiento farmacológico , Niño , Endocarditis Bacteriana/diagnóstico , Endocarditis Bacteriana/tratamiento farmacológico , Endocarditis Bacteriana/cirugía , Femenino , Humanos , Infecciones Estafilocócicas/diagnóstico , Infecciones Estafilocócicas/tratamiento farmacológicoRESUMEN
A male infant with Kabuki syndrome had trivial congenital mitral regurgitation diagnosed at birth. At the age of 2 years and 9 months, the regurgitation worsened from mild to severe; thus, expedited surgical treatment was pursued. The primary operative finding was severe dysplastic two-leaflet disease. After completing chordal replacement as a conventional repair procedure, more-than-moderate central regurgitation caused by establishing a shallow coaptation between the anterior and posterior leaflets persisted. We report a successful case of mitral valve repair involving the novel option of interannular bridge for valvuloplasty to address congenital mitral regurgitation.
Asunto(s)
Cara/anomalías , Enfermedades Hematológicas/complicaciones , Anuloplastia de la Válvula Mitral , Insuficiencia de la Válvula Mitral/congénito , Insuficiencia de la Válvula Mitral/cirugía , Enfermedades Vestibulares/complicaciones , Anomalías Múltiples , Preescolar , Humanos , Masculino , Insuficiencia de la Válvula Mitral/diagnóstico por imagenRESUMEN
OBJECTIVES: Patients with unbalanced pulmonary artery (PA) growth and decreased unilateral pulmonary circulation are considered unsuitable candidates for the Fontan procedure. Following our previous study on the utility of intrapulmonary-artery septation for patients with PA hypoplasia, we investigated its use in patients with pulmonary venous obstruction (PVO). METHODS: We recruited 42 patients who underwent intrapulmonary-artery septation for unilateral PA hypoplasia and/or PVO between 1998 and 2018 and classified them into no PVO or PVO group. We analysed overall survival, success of the Fontan procedure and data from catheterization and echocardiography. In PVO, we evaluated the functional lung area before the Fontan procedure and the relevance of this parameter to operative outcomes. RESULTS: The PVO and no-PVO group included 24 and 18 patients, respectively. One patient in the no-PVO and 6 patients in the PVO group died during follow-up; this difference was statistically significant (log-rank P = 0.040). In the no-PVO group, 15 (83%) patients achieved two-lung Fontan circulation. In the PVO group, 12 (50%) patients achieved two-lung Fontan circulation. Multivariate analysis revealed that functional lung area and shunt size [significantly larger in patients with functional lung area ≥50% in affected lung (P = 0.040)] were significant factors for successful two-lung Fontan procedure (P = 0.030). CONCLUSIONS: Intrapulmonary-artery septation may contribute to increase functional lung area after PVO release to establish two-lung Fontan circulation in patients with PVO who have unbalanced PA growth and/or decreased unilateral pulmonary circulation.
Asunto(s)
Procedimiento de Fontan , Cardiopatías Congénitas , Venas Pulmonares , Procedimiento de Fontan/efectos adversos , Cardiopatías Congénitas/diagnóstico por imagen , Cardiopatías Congénitas/cirugía , Humanos , Pulmón , Arteria Pulmonar/diagnóstico por imagen , Arteria Pulmonar/cirugía , Circulación Pulmonar , Venas Pulmonares/diagnóstico por imagen , Venas Pulmonares/cirugíaRESUMEN
A boy with 22q11.2 deletion was diagnosed with pulmonary atresia with ventricular septal defect (PAVSD) and major aortopulmonary collateral arteries (MAPCAs). At 8 months, unifocalization of left MAPCAs and BT shunt was performed at another hospital. However, they occluded directly after surgery. An angiography revealed 2 MAPCAs supplying all segments of the right lung. Qp:Qs was 2.24. At 1 year 6 months, we performed VSD closure with unifocalization of the only right lung vascularity, using intraoperative PA flow study. We report a successful case of complete repair with unilateral lung in PAVSD and MAPCAs.
Asunto(s)
Aorta/cirugía , Defectos de los Tabiques Cardíacos/cirugía , Pulmón/irrigación sanguínea , Arteria Pulmonar/cirugía , Atresia Pulmonar/cirugía , Aorta/anomalías , Procedimiento de Blalock-Taussing , Circulación Colateral , Síndrome de DiGeorge/complicaciones , Defectos de los Tabiques Cardíacos/complicaciones , Humanos , Lactante , Recién Nacido , Masculino , Arteria Pulmonar/anomalías , Atresia Pulmonar/complicacionesRESUMEN
OBJECTIVES: To evaluate the clinical outcomes of surgical repair of an extracardiac total anomalous pulmonary venous connection (TAPVC) in a functional single-ventricle (f-SV) strategy. METHODS: This was a retrospective analysis of 48 consecutive cases of extracardiac TAPVC repair, from 1998 to 2015. Demographic and clinical variables were as follows: median age, 24 (range 0-744) days; median weight, 3.1 (range 2.0-9.6) kg; type of TAPVC-supracardiac, 21 patients, infracardiac, 8, and mixed, 19; right atrial isomerism, 45 patients; pulmonary atresia, 24 patients; and obstructed TAPVC, 30 patients. Concomitant procedures included systemic-to-pulmonary shunting in 15 patients, pulmonary artery banding in 12, ventricle-to-pulmonary artery shunting in 2, a Norwood procedure in one, a bidirectional Glenn procedure in 16, and a Fontan procedure in 1. RESULTS: The 1- and 5-year cumulative survival rates were 66.0% and 58.0%, respectively. Of the 28 survivors, 22 (78.6%) underwent Fontan completion and 4 (14.3%) a bidirectional Glenn procedure, and 1 (3.6%) was awaiting a bidirectional Glenn procedure. Recurrent pulmonary venous stenosis (PVS) was observed in 17 patients, with a 1- and 5-year rates of freedom from recurrent PVS 59.8% and 53.5%, respectively. Of the 20 post-operative deaths, only 4 were PVS related. Only pre-operative pulmonary atresia was identified as an independent risk factor of mortality after TAPVC repair. CONCLUSIONS: The midterm surgical outcomes of f-SV with extracardiac TAPVC were acceptable. Moreover, among survivors, Fontan completion can be sufficiently expected. Further improvement, with the development of a comprehensive treatment strategy, is required for this patient group.
Asunto(s)
Procedimientos Quirúrgicos Cardiovasculares/métodos , Síndrome de Heterotaxia/cirugía , Atresia Pulmonar/cirugía , Venas Pulmonares/cirugía , Síndrome de Cimitarra/cirugía , Preescolar , Femenino , Procedimiento de Fontan , Humanos , Lactante , Recién Nacido , Masculino , Procedimientos de Norwood , Arteria Pulmonar/anomalías , Arteria Pulmonar/cirugía , Venas Pulmonares/anomalías , Recurrencia , Estudios Retrospectivos , Factores de Riesgo , Estenosis de Vena Pulmonar/etiología , Tasa de Supervivencia , Factores de Tiempo , Corazón Univentricular/cirugíaRESUMEN
An infant boy with 21 trisomy was diagnosed with complete atrioventricular septal defect-Rastelli type A with parachute left atrioventricular valve and absent left mural leaflet. Biventricular repair was difficult in infancy because the left ventricular volume and left atrioventricular valve annulus were too small. After 2 pulmonary artery bandings, the left atrioventricular valve annulus increased to 85% of normal mitral valve, and the left ventricular end-diastolic volume increased to 98% of normal. We report a successful instance of complete atrioventricular septal defect that achieved biventricular repair via novel left atrioventricular valvuloplasty for parachute left atrioventricular valve and absent left mural leaflet.
Asunto(s)
Valvuloplastia con Balón/métodos , Síndrome de Down/diagnóstico , Ecocardiografía Transesofágica/métodos , Defectos de los Tabiques Cardíacos/diagnóstico por imagen , Defectos de los Tabiques Cardíacos/cirugía , Válvula Mitral/cirugía , Procedimientos Quirúrgicos Cardíacos/métodos , Síndrome de Down/complicaciones , Ecocardiografía/métodos , Estudios de Seguimiento , Humanos , Recién Nacido , Masculino , Válvula Mitral/diagnóstico por imagen , Arteria Pulmonar/cirugía , Recuperación de la Función/fisiología , Resultado del TratamientoRESUMEN
OBJECTIVES: Extensive clinicopathological analyses of aortic dissection have implicated hypertension and genetic abnormalities as the major pathogenic mechanisms. However, previous findings from pathological examinations have often been inconsistent with these mechanisms. In this paper, we suggest a significant role for the vasa vasorum in the aetiology and pathogenesis of aortic dissection. METHODS: We reviewed records of patients who underwent thoracic aortic dissection repair at our institution between June 2008 and August 2011. Twenty-one patients (10 men, 11 women; mean age, 65.0 ± 12.0 years) underwent surgery with subsequent histopathological examination of the aortic wall. We evaluated the history and histopathological findings of these patients. RESULTS: Aortic medial changes were observed in all 21 patients. These changes included thinning and sometimes fragmentation of the elastic lamina, as well as atrophy of the smooth muscle cells, with surrounding accumulation of an Alcian blue-positive mucinous substance, mostly adjacent to the dissection. Importantly, the dissection was located in the outer third of the media in 20 of the 21 patients (95.2%). Of these 20, 18 showed histopathological evidence of sclerotic changes of the vasa vasorum, including muscular hyperplasia, elastosis, intimal fibrosis and/or luminal obstruction, and even rupture. These changes may have been secondary to hypertension or peri-aortic changes, leading to degenerative changes in the aortic media and even initiation of dissection. CONCLUSIONS: Most aortic dissections initially developed in the outer third of the media alongside the vasa vasorum. In this type of aortic dissection, dysfunction of the vasa vasorum may play a key role in long-standing ischaemia or malnutrition of the aortic media.