Prognostic markers in congenital diaphragmatic hernia: Left ventricular
diameter and pulmonary hypertension
and Olgu Hallioglu
Department of Pediatrics, Division of Pediatric Cardiology and
Department of Pediatrics, Division of Neonatology,
Faculty of Medicine, University of Mersin, Mersin, Turkey
Abstract Background: The aim of this study was to investigate whether the outcome of congenital diaphragmatic hernia
(CDH) in newborns can be predicted using left ventricular (LV) diameter and estimated pulmonary artery pressure.
Methods: Patients in the newborn unit in 2012–2016 were screened retrospectively. Echocardiographic measurements
of 35 patients with isolated left lateral CDH and 27 healthy newborns were compared in the ﬁrst 24 h of life.
Results: Mean LV end-diastolic diameter (LVEDD) and end-systolic diameter (LVESD), and LV ejection fraction
were signiﬁcantly lower in the CDH group. Moreover, tricuspid regurgitation (TR) and pulmonary regurgitation
(PR) were signiﬁcantly higher than in the controls (P < 0.001). Six CDH patients died within the next 40 days after
birth. Mean LVEDD and LVESD were signiﬁcantly lower in the CHD patients who died compared with those who
were discharged (P < 0.001, P = 0.016). Also, mean TR and PR (P < 0.001) and the frequency of pulmonary
hypertension (PH; P = 0.001) were signiﬁcantly higher in these patients. On receiver operating characteristic
analysis of the CDH non-survivors, LVEDD < 11 mm (sensitivity, 100%; 95%CI: 87.9–100; speciﬁcity, 100%;
95%CI: 54.1–100) and TR > 3.5 m/s (sensitivity, 89.66%; 95%CI: 72.6–97.7; speciﬁcity, 100%; 95%CI: 54.1–100)
were associated with poor prognosis.
Conclusions: Decrease in LVEDD and presence of PH are associated with poor prognosis. Also, PH was associated
with mortality in CDH patients. Thus, outcome may be predicted on careful echocardiographic evaluation of the
LV diameters and pulmonary pressure.
Key words congenital diaphragmatic hernia, echocardiography, pulmonary hypertension.
Despite improvements in intensive care support, congenital
diaphragmatic hernia (CDH) is still a life-threatening anomaly.
The prevalence of the disease is 1:2,000–1:3,000 live births and
the mortality rate is high (30–40%).
Although many factors
play role in the prognosis of CDH, the major determinant of
survival is pulmonary hypoplasia and related pulmonary
Displacement of abdominal organs to the thoracic cavity
due to the diaphragm defect causes pulmonary hypoplasia and
abnormality in pulmonary vascular structure by affecting lung
development in the intrauterine period. The degree of PH as a
result of abnormal heart and lung development determines the
severity and prognosis of CDH.
Progressive increase in
pulmonary artery pressure (PAP), right ventricular (RV) failure
and concomitant left ventricular (LV) dysfunction are associated
with mortality in PH.
The degree of PH and persistent PH
(PPH) can be determined on conventional echocardiography.
There are limited studies on the echocardiographic prognostic
indices of CDH in infants. The aim of this study was therefore
to investigate whether LV diameter and non-invasive predicted
PAP could be used to predict outcome in CDH.
This retrospective study was performed in January 2012–
December 2016 in patients with CDH. The exclusion crite-
ria were birth before 36 weeks of gestation, presence of
severe intrauterine growth retardation, severe cardiac or con-
genital malformations and incomplete echocardiography. The
study included 35 patients with isolated left-sided CDH.
The medical and demographic data and echocardiographic
records of the CDH patients were obtained from medical
records. Echocardiography was performed in the ﬁrst 24 h
after birth for assessment of myocardial involvement. The
control group consisted of healthy neonates with normal
echocardiography and electrocardiography, and no history of
cardiovascular disease. The local ethics committee provided
At the present hospital, newborns with an antenatal
diagnosis of CDH and respiratory distress are immediately
intubated after birth. In the neonatal intensive care unit
Correspondence: Derya Karpuz, MD, Department of Pediatric
Cardiology, Faculty of Medicine, Mersin University, TR-33343,
Mersin, Turkey. Email: email@example.com
Received 18 July 2017; revised 17 October 2017; accepted 22
© 2017 Japan Pediatric Society
Pediatrics International (2018) 60, 122–126 doi: 10.1111/ped.13464