Repair of Total Anomalous Pulmonary Venous Connection: Risk Factors for Postoperative Obstruction

Abstract

Background.-Pulmonary venous obstruction after repair of total anomalous pulmonary venous connection (TAPVC) results in substantial morbidity and mortality. Risk factors for postoperative obstruction remain ambiguous. Additionally, the existing literature has no standard definition for preoperative obstruction making patient counseling difficult. Methods.-All patients undergoing repair of TAPVC at our institution from 1/1/06 to 10/23/17 were identified. The primary outcome was the development of postoperative obstruction, analyzed as a time-to-event outcome. Clinical information was extracted to assess risk factors. Degrees of preoperative obstruction were defined based on echocardiographic, catheterization, and clinical findings. Univariable and multivariable Cox proportional hazard regression methods were used to identify factors associated with the primary outcome. Results.-During the study interval, 119 patients underwent repair of TAPVC (40% singleventricle), and 25 patients (21%) developed postoperative obstruction. Risk factors associated with obstruction were heterotaxy syndrome, single-ventricle heart disease, additional surgeries at the time of vein repair, mixed-type TAPVC, and preoperative obstruction. Having even mild preoperative obstruction (≥1.2 m/s by Doppler echocardiography) was predictive of postoperative obstruction. A multivariable model showed mixed-type TAPVC and the presence of preoperative obstruction were associated with over 2-fold greater hazards of obstruction. Conclusions.-TAPVC in the setting of heterotaxy and single-ventricle remains challenging with high rates of postoperative obstruction. Mixed-type TAPVC is an independent risk factor for postoperative obstruction, particularly in patients with isolated TAPVC. Even mild preoperative obstruction is a risk factor for postoperative obstruction. These results may help risk-stratify TAPVC patients.

Commentary: Relentless disease, relentless measures: Treat recurrent pulmonary vein obstruction early and often

“…As reported previously, 5,6 recurrent vein disease predominantly presented within the first year after surgery. Similar to another study that identified heterotaxy, a single ventricle, and primary obstruction as risk factors for recurrent obstruction, 6 this report showed primary obstruction with either heterotaxy or a single ventricle to be associated with increased pulmonary vein interventions. Notably, the need for pulmonary vein intervention was a marker for decreased survival.…”

Secondary Pulmonary Vein Stenosis Due to Total Anomalous Pulmonary Venous Connection Repair in Children: Extravascular MDCT Findings

“…PVS is mainly classified into two types: primary and secondary [ 1 , 2 ]. While primary PVS occurs in the absence of previous intervention, secondary PVS is known to develop following total anomalous pulmonary venous connection (TAPVC) repair with an incidence of 11–17% [ 1 , 2 , 3 , 4 , 5 , 6 ]. Affected pediatric patients typically present with non-specific signs and symptoms, such as shortness of breath, failure to thrive, hypoxemia, pulmonary hypertension (HTN), and fatigue [ 1 , 2 , 3 , 4 , 5 , 6 ].…”

“…While primary PVS occurs in the absence of previous intervention, secondary PVS is known to develop following total anomalous pulmonary venous connection (TAPVC) repair with an incidence of 11–17% [ 1 , 2 , 3 , 4 , 5 , 6 ]. Affected pediatric patients typically present with non-specific signs and symptoms, such as shortness of breath, failure to thrive, hypoxemia, pulmonary hypertension (HTN), and fatigue [ 1 , 2 , 3 , 4 , 5 , 6 ]. Mainly due to its rarity and non-specific clinical presentation, PVS diagnosis is often missed or delayed, resulting in suboptimal patient care.…”

Pulmonary Vein Stenosis—Evolving Surgical Management of a Challenging Disease

“…Overall mortality may be better in patients undergoing the primary repair of total anomalous pulmonary venous connection (TAPVC) with contemporary three-year survival rates approximating 85% [7]. Nonetheless, post-repair pulmonary vein obstruction occurs in up to 15-21% of these patients [8,9] and remains an ongoing challenge with survival rates of only 58-73% [10,11]. This review will discuss the evolution of PVS surgery, with a particular emphasis on the principles underlying contemporary surgical techniques and newer repair strategies.…”