Persistent Pulmonary Hypertension of the Newborn in Twin-Twin Transfusion Syndrome: A Case-Control Study

Neonatology. 2017;112(4):402-408. doi: 10.1159/000478844. Epub 2017 Sep 20.

Abstract

Background: Persistent pulmonary hypertension of the newborn (PPHN) is associated with severe morbidity and mortality. Twin-twin transfusion syndrome (TTTS) is suggested to increase the risk of PPHN.

Objectives: To describe the incidence of PPHN in TTTS twins and to identify risk factors in TTTS twins for the development of severe PPHN.

Methods: Cases with severe PPHN were extracted from our monochorionic twin database (2002-2016). Severe PPHN was defined as severe hypoxaemia requiring mechanical ventilation and inhaled nitric oxide (iNO) treatment, confirmed by strict echocardiographic criteria. A case-control comparison within TTTS survivors was conducted to identify risk factors for PPHN.

Results: The incidence of PPHN in TTTS twins was 4% (24/598, 95% confidence interval [CI] 2.7-5.9%) and 0.4% (2/493, 95% CI 0.1-1.5%) in uncomplicated monochorionic twins (odds ratio [OR] 10.3, 95% CI 2.4-43.9; p = 0.002). Two risk factors were independently associated with PPHN: severe prematurity (OR 3.3, 95% CI 1.0-11.4) and recipient status (OR 3.9, 95% CI 1.4-11.0). In TTTS recipients, another risk factor for PPHN is anaemia at birth (OR 7.2, 95% CI 1.8-29.6).

Conclusion: Clinicians caring for neonates with TTTS should be aware of the 10-fold increased risk of PPHN compared to uncomplicated monochorionic twins. PPHN occurs more often in case of premature delivery and in recipient twins, particularly in the presence of anaemia at birth. As the development of severe PPHN is difficult to predict, we advise that all TTTS twins should be delivered in a tertiary care centre with iNO treatment options.

Keywords: Case-control study; Fetoscopic laser surgery; Monochorionic twin pregnancy; Persistent pulmonary hypertension of the newborn; Recipient; Twin-twin transfusion syndrome.

Publication types

  • Twin Study

MeSH terms

  • Administration, Inhalation
  • Case-Control Studies
  • Databases, Factual
  • Female
  • Fetofetal Transfusion / diagnosis
  • Fetofetal Transfusion / epidemiology*
  • Fetofetal Transfusion / physiopathology
  • Fetofetal Transfusion / therapy
  • Humans
  • Incidence
  • Logistic Models
  • Multivariate Analysis
  • Netherlands / epidemiology
  • Nitric Oxide / administration & dosage
  • Odds Ratio
  • Persistent Fetal Circulation Syndrome / diagnosis
  • Persistent Fetal Circulation Syndrome / epidemiology*
  • Persistent Fetal Circulation Syndrome / physiopathology
  • Persistent Fetal Circulation Syndrome / therapy
  • Pregnancy
  • Respiration, Artificial
  • Risk Factors
  • Severity of Illness Index
  • Time Factors
  • Twins, Monozygotic*
  • Vasodilator Agents / administration & dosage

Substances

  • Vasodilator Agents
  • Nitric Oxide