The unnatural history of pulmonary stenosis up to 40 years after surgical repair

Heart. 2017 Feb 15;103(4):273-279. doi: 10.1136/heartjnl-2015-309159. Epub 2016 Aug 11.

Abstract

Objective: To provide prospective information on long-term outcome after surgical correction of valvular pulmonary stenosis (PS).

Methods: Fifty-three consecutive patients operated for PS during childhood between 1968 and 1980 in one centre are followed longitudinally for 37±3.4 years, including extensive in-hospital examination every 10 years.

Results: Survival information was available in 100% of the original 53 patients. Cumulative survival was 94% at 20 years and 91% at 40 years. Excluding perioperative mortality (<30 days), survival was 94% at 40 years. Of 46 eligible survivors, 29 participated in the in-hospital examination and 15 gave permission to use their hospital records (96% participation). Cumulative event-free survival was 68% after 40 years: 25% needed a reintervention, 12% underwent pacemaker implantation and 9% had supraventricular arrhythmias. Early reinterventions were mainly for residual PS, late reinterventions for pulmonary regurgitation. Subjective health status was good. Exercise capacity was normal in 74% (median 96 (82-107)% of expected workload). Right ventricular and left ventricular (LV) dysfunction was found in 13% and 41%, respectively. The use of a transannular patch and younger age at surgery were predictive for late events (HR 3.02 (95% CI 1.09 to 8.37) and HR 0.81/year (95% CI 0.66 to 0.98), respectively). Use of inflow occlusion compared with cardiopulmonary bypass showed a trend towards more reinterventions (HR 3.19 (95% CI 0.97 to 10.47)).

Conclusions: Survival up to 40 years after successful PS repair is nearly normal. Subjective health status is good and there is a low incidence of arrhythmias. Reinterventions, however, are necessary in one-quarter and 40 years postoperatively several patients show LV dysfunction.

Publication types

  • Research Support, Non-U.S. Gov't

MeSH terms

  • Adolescent
  • Adult
  • Arrhythmias, Cardiac / etiology
  • Arrhythmias, Cardiac / physiopathology
  • Arrhythmias, Cardiac / therapy
  • Biomarkers / blood
  • Cardiac Surgical Procedures* / adverse effects
  • Cardiac Surgical Procedures* / mortality
  • Disease-Free Survival
  • Echocardiography
  • Electrocardiography, Ambulatory
  • Exercise Test
  • Exercise Tolerance
  • Female
  • Health Status
  • Humans
  • Kaplan-Meier Estimate
  • Longitudinal Studies
  • Male
  • Middle Aged
  • Natriuretic Peptide, Brain / blood
  • Netherlands
  • Peptide Fragments / blood
  • Proportional Hazards Models
  • Prospective Studies
  • Pulmonary Valve / physiopathology
  • Pulmonary Valve / surgery*
  • Pulmonary Valve Stenosis / diagnosis
  • Pulmonary Valve Stenosis / mortality
  • Pulmonary Valve Stenosis / physiopathology
  • Pulmonary Valve Stenosis / surgery*
  • Recovery of Function
  • Retreatment
  • Risk Factors
  • Time Factors
  • Treatment Outcome
  • Ventricular Dysfunction, Left / etiology
  • Ventricular Dysfunction, Left / physiopathology
  • Ventricular Dysfunction, Left / therapy
  • Ventricular Dysfunction, Right / etiology
  • Ventricular Dysfunction, Right / physiopathology
  • Ventricular Dysfunction, Right / therapy
  • Ventricular Function, Left
  • Ventricular Function, Right
  • Young Adult

Substances

  • Biomarkers
  • Peptide Fragments
  • pro-brain natriuretic peptide (1-76)
  • Natriuretic Peptide, Brain