Ebstein's anomaly: "The one and a half ventricle heart"

Abstract Objective: Ebstein's anomaly remains a relatively ignored disease. Lying in the 'No Man's land' between congenital and valve surgeons, it largely remains inadequately studied. We report our short-term results of treating it as a 'one and a half ventricle heart' and propose that the true tricuspid annulus (TTA) 'Z' score be used as an objective criterion for estimation of 'functional' right ventricle (RV). Methods: 22 consecutive patients undergoing surgery for Ebstein's anomaly were studied. Echocardiography was performed to assess the type and severity of the disease, tricuspid annular dimension and its 'Z' score. Patients were operated by a modification of the cone repair, with addition of annuloplasty, bidirectional cavopulmonary shunt (BCPS) and right reduction atrioplasty to provide a comprehensive repair. TTA 'Z' score was correlated later with postplication indexed residual RV volume. Results: There was one (4.5%) early and no late postoperative death. There was a significant reduction in tricuspid regurgitation grading (3.40±0.65 to 1.22±0.42, P<0.001). Residual RV volume reduced to 71.96±3.8% of the expected volume and there was a significant negative correlation (rho −0.83) between TTA 'Z' score and indexed residual RV volume. During the follow-up of 20.54±7.62 months, the functional class improved from 2.59±0.7 to 1.34±0.52 (P<0.001). Conclusion: In Ebstein's anomaly, a higher TTA 'Z' score correlates with a lower postplication indexed residual RV volume. Hence, a complete trileaflet repair with offloading of RV by BCPS (when the TTA 'Z' score is >2) is recommended. The short-term outcomes of our technique are promising.

Reparación de la insuficiencia tricuspídea: resultados a largo plazo en 114 pacientes / Repair of tricuspid valve insufficiency: long-term results in 114 patients

Resumen Antecedentes la reparación de la insuficiencia tricuspídea funcional está indicada en casos de insuficiencia cardíaca, generalmente, asociada a enfermedades de las válvulas cardíacas izquierdas y en algunos casos de enfermedad coronaria o cardiopatías congénitas. Objetivo : evaluar los resultados a largo plazo de la reparación tricuspídea en pacientes operados en la región centro sur de Chile. Pacientes y métodos : estudio retrospectivo de registros clínicos y operatorios en 114 pacientes con edad promedio 57.8 (DE 13) años (72 mujeres) sometidos a reparación tricuspídea asociada a otros procedimientos quirúrgicos entre 2009 y 2017. Resultados : la etiología de la insuficiencia tricuspídea fue debida a enfermedad reumática inactiva en 45% y a endocarditis infecciosa en 2.6%. 63% estaban en fibrilación auricular y 75% en capacidad funcional NYHC III. 15.8% tenían cirugía valvular cardíaca previa. El ecocardiograma mostró insuficiencia severa en 56% de los casos y había hipertensión pulmonar severa en 39.5%. La cirugía consistió en la implantación de un anillo semi-rígido en forma concomitante a reemplazo o reparación de lesión valvular mitral, reemplazo valvular aórtico, cierre de comunicación interauricular, cirugía coronaria o resección de tumor intra cardíaco. La mortalidad post operatoria global fue 16%, debido a falla multisistémica en 6%, insuficiencia cardíaca en 5% y hemorragia cerebral en 4.3%. El seguimiento promedio fue 78.8 (DS 7.2) meses. La supervivencia actuarial fue 74% a los 60 y 68% a los 96 meses. Conclusión : La cirugía de reparación de la insuficiencia tricuspídea moderada o severa, asociada a otras enfermedades cardíacas provee una buena recuperación sintomática, con excelente sobrevida alejada.

Abstract Background : Surgery for functional tricuspid valve insufficiency is indicated in cases of congestive heart failure usually associated to left heart valve diseases or as a concomitant surgery for other causes such as congenital or coronary disease. Aim : To assess and report the long-term results of tricuspid valve repair in adult patients in a regional cardiac surgery center in south Chile. Patients and Methods : Retrospective review of clinical and operative records of 114 patients aged 57.8 +/- 13 years (72 women) subjected to tricuspid reparative surgery concomitant with other cardiac procedures between 2009 to 2017. Results : In 45% of cases etiology was due to inactive rheumatic disease and 2.6% was due to endocarditis. 63% presented with atrial fibrillation and 75% were in NYHC CFIII. 15.8% had a previous cardiac valve surgery. Preoperative echocardiography showed severe tricuspid insufficiency in 56% of cases and pulmonary hypertension was severe in 39.5%. In all cases tricuspid repair was performed through the insertion of a semi rigid ring as a concomitant procedure for mitral repair/replacement in most cases, aortic valve replacement, surgical closure of an ASD, CABG surgery and the resection of cardiac tumors. Overall postoperative mortality was 16% due to multi-organic dysfunction in 6%, cardiac failure in 5% and cerebral hemorrhage in 4.3%. Mean long term follow up was 78.8+/- 7.2 months. Actuarial survival was 74% at 60 and 68% at 96 months. Conclusion: Surgical tricuspid valve repair for moderate to severe tricuspid insufficiency isolated or associated to other cardiac diseases provides a good symptomatic recovery, with an excellent long term survival.

Morbimortalidade em pacientes acima de 75 anos submetidos à cirurgia por estenose valvar aórtica / Morbidity and mortality in patients aged over 75 years undergoing surgery for aortic valve replacement

FUNDAMENTO: A maior longevidade observada atualmente ocasionou aumento do número de idosos que necessitam de intervenções cirúrgicas. A estenose aórtica é uma condição frequente nessa faixa etária. OBJETIVO: Avaliar morbidade e mortalidade hospitalar em pessoas de 75 anos ou mais, que tenham sido submetidas à cirurgia de valvuloplastia, ou de troca valvar por estenose aórtica isolada ou associada a outras lesões. MÉTODOS: Foram estudados 230 casos consecutivos entre jan/2002-dez/2007. Os pacientes tinham 79,5 ± 3,7 anos (75 - 94), sendo que 53,9 por cento eram homens. Na amostra, 68,7 por cento tinham hipertensão arterial, 17,9 por cento tinham fibrilação atrial, 15,9 por cento apresentaram obesidade e 14,4 por cento cirurgia cardíaca prévia. Na cirurgia, 87,4 por cento foram submetidos à colocação de prótese aórtica e 12,6 por cento à valvuloplastia aórtica. RESULTADOS: A mortalidade foi de 13,9 por cento (sendo 9,4 por cento de estenose aórtica isolada x 20,9 por cento com procedimento associado; p = 0,023) e a morbidade foi de 30,0 por cento (sendo 25,2 por cento de estenose aórtica isolada x 37,4 por cento com procedimento associado; p = 0,068). As complicações mais frequentes foram: baixo débito cardíaco (20,2 por cento), disfunção renal (9,7 por cento) e suporte ventilatório prolongado (7,9 por cento). Na análise bivariada, os maiores preditores de mortalidade foram: baixo débito cardíaco (RR 10,1, IC95 por cento: 5,02-20,3), uso do balão intra-aórtico (RR 6,6, IC95 por cento: 3,83-11,4), sepse (RR 6,77, IC95 por cento: 1,66-9,48) e disfunção renal pós-operatória (RR 6,21, IC95 por cento: 3,47-11,1). Quanto à morbidade, foram preditores: disfunção renal pré-operatória (RR 2,22, IC95 por cento: 1,25-3,95), fibrilação atrial (RR 1,74, IC95 por cento: 1,16-2,61) e doença pulmonar obstrutiva crônica (DPOC) (RR 1,93, IC95 por cento: 1,25-2,97). CONCLUSÃO: A cirurgia valvar aórtica em idosos está relacionada à morbimortalidade um pouco ...

BACKGROUND: The greater longevity observed today has caused an increase in the number of elderly who need surgery. Aortic stenosis is a common condition in this age group. OBJECTIVE: To evaluate morbidity and mortality in people aged 75 years or older who have undergone valvuloplasty or valve replacement surgery for aortic stenosis alone or associated with other injuries. METHODS: We studied 230 consecutive cases between Jan/2002-Dec/2007. Patients were 79.5 ± 3.7 years (75 - 94), and 53.9 percent were men. In the sample, 68.7 percent had hypertension, 17.9 percent had atrial fibrillation, 15.9 percent were obese, and 14.4 percent had undergone previous heart surgery. At surgery, 87.4 percent underwent aortic stent placement, and 12.6 percent underwent aortic valvuloplasty. RESULTS: The mortality rate was 13.9 percent (9.4 percent with isolated aortic stenosis versus 20.9 percent with an associated procedure, p = 0.023) and the morbidity rate was 30.0 percent (25.2 percent with aortic stenosis alone versus 37.4 percent with an associated procedure, p = 0.068). The most common complications were: low cardiac output (20.2 percent), renal dysfunction (9.7 percent), and prolonged ventilatory support (7.9 percent). In the bivariate analysis, the main predictors of mortality were low cardiac output (RR 10.1, 95 percent CI: 5.02-20.3), use of intra-aortic balloon (RR 6.6, 95 percent CI: 3.83-11.4), sepsis (RR 6.77, 95 percent: 1.66-9.48) and renal dysfunction after surgery (RR 6.21, 95 percent: 3.47-11.1). As for morbidity, the predictors were: pre-operative renal dysfunction (RR 2.22, 95 percent: 1.25-3.95), atrial fibrillation (RR 1.74, 95 percent: 1.16-2.61), and chronic obstructive pulmonary disease (COPD) (RR 1.93, 95 percent: 1.25-2.97). CONCLUSION: Aortic valve surgery in the elderly is related to a slightly higher mortality rate than in younger patients, and its main risk factors were associated procedures, renal failure, atrial fibrillation, ...