Pulmonary atresia with intact ventricular septum: Long-term results of “One and a Half Ventricular Repair”

Kim

European Journal of Cardio-Thoracic Surgery

2011

The patients with one-and-a-half ventricle repair resulted in favorable late survival in this series. During the follow-up period, most surviving patients showed good functional status without common late complications of the Fontan procedure such as, recurrent cyanosis, pulmonary arteriovenous fistulas, chronic arrhythmias, and SVC syndrome. This procedure appears to be a valid alternative to Fontan and biventricular repairs in patients with right-ventricular dysfunction or hypoplasia.

Section: Discussionmentioning

confidence: 89%

Section: Discussionmentioning

confidence: 98%

Long-term results of one-and-a-half ventricle repair in complex cardiac anomalies

Kim

European Journal of Cardio-Thoracic Surgery

2011

Rev Bras Cir Cardiovasc

“…The major concern in the extended application of one and a half ventricular repair is the currently limited knowledge of the long term results, due to the relatively recently utilization of this surgical approach and to the reduced number of clinical reports in this regard [18].…”

Section: Discussionmentioning

confidence: 99%

One and a half ventricular repair as an alternative for hypoplastic right ventricle

Maluf¹,

Carvalho²,

Carvalho³

2010

Operação de um ventrículo e meio como uma alternativa para o ventrículo direito hipoplásicoOne and a half ventricular repair as an alternative for hypoplastic right ventricle Endereço Conclusões: O tratamento cirúrgico de cardiopatias congênita, na presença de um ventrículo direito hipoplásico, por meio da operação de um ventrículo e meio, tem menor risco cirúrgico que a correção biventricular, mantendo o átrio direito com baixa pressão, fluxo sanguíneo pulmonar pulsátil e melhora da saturação sistêmica de oxigênio. Os resultados de curto e médio prazo são satisfatórios; porém será necessário um maior tempo de seguimento para provar a eficácia desta abordagem cirúrgica a longo prazo. Descritores 467MALUF, MA ET AL -Operação de um ventrículo e meio como uma alternativa para o ventrículo direito hipoplásico Bras Cir Cardiovasc 2010; 25(4): 466-473 Conclusions: Surgical treatment of congenital cardiac anomalies in the presence of a hypoplastic right ventricle by means of one and a half ventricle repair has the advantages of reducing the surgical risk of biventricular repair compared to the Fontan circulation; it maintains a low right atrium pressure, a pulsatile pulmonary blood flow and improves the systemic oxygen saturation with short and medium-term promising results. Longer follow-up is needed to prove the efficacy of such a repair in the long term. RevDescriptors: Heart defects, congenital/surgery. Heart valve prosthesis implantation, methods. Child. Pulmonary atresia. Ebstein anomaly.

Gen Thorac Cardiovasc Surg

“…They performed a BT shunt simultaneously with transpulmonary valvotomy in patients whose RVDI was <0. 35. They indicated that neither the RV end-diastolic volume nor the TV diameter could be a reliable indicator in isolation for selecting the initial palliative procedure and concluded that the RVDI therefore provided suffi cient criteria for the initial management strategy for patients with PA-IVS.…”

Section: Initial Palliations and Subsequent Interventionsmentioning

confidence: 96%

Surgical strategy for pulmonary atresia with intact ventricular septum: initial management and definitive surgery

Yoshimura

Yamaguchi

2009

Pulmonary atresia with intact ventricular septum is difficult to manage, and the surgical procedures are associated with high mortality and morbidity. The lesion is morphologically heterogeneous, with varying degrees of right ventricular and tricuspid valve hypoplasia. Aberrations of the coronary circulation are common. A single approach is impractical because the wide spectrum of right heart morphology makes a uniform surgical approach impossible. Anatomical criteria, such as the degree of tricuspid valve or right ventricular hypoplasia or whether a right ventricular infundibulum is present, have been used to assign the infants to single-ventricular or biventricular repair tracks. If the patient is considered a candidate for biventricular repair, right ventricular decompression, either alone or combined with a systemic-pulmonary artery shunt, is performed, whereas only a systemic-pulmonary artery shunt is performed in a candidate for single-ventricular repair. Several authors reported that the Z-value of the tricuspid valve diameter could be a guide to the initial procedure; however, poor interobserver reproducibility and wide normal ranges are serious limitations. Definitive operations, including biventricular repair, one-and-a-half ventricular repair, or Fontan-type operation, are performed to eliminate cyanosis. The most important problem when planning a strategy for a definitive repair is the fact that right ventricular growth and its contribution to pulmonary flow is difficult to predict before the operation. The management protocol should be determined by the precisely estimated morphology of the right ventricle and tricuspid valve, and the adequacy of the protocol should be analyzed based on surgical outcomes.