Pulmonary Hypertension Screening Adaptations for Adult Congenital Heart Disease Subtypes

Transposition of the great arteries after atrial redirection

Patients with the Mustard or Senning procedures cannot be screened for PH with the TR gradient, so clinicians should consider PH when:

  • The following parameters are present: PR and MR gradients, increased size of the PAs, and the forward flow across the pulmonary valve
  • The subpulmonary left ventricle shows progressive dilation

“Current international PH guidelines describe the echocardiographic criteria that should be used to identify patients with possible PH, but these do not apply to many patients [with congenital heart disease]; for example, those with univentricular hearts, systemic right ventricles, pulmonary stenosis, or atresia,” explained lead author Konstantinos Dimopoulos, MD, MSc, PhD, from the Royal Brompton and Harefield NHS Foundation Trust and the Adult Congenital Heart Centre and Centre for Pulmonary Hypertension in London, United Kingdom, in an interview with Pulmonology Advisor. “Even though evidence is limited, it is essential that physicians and echocardiography experts understand the anatomy and physiology of [congenital heart disease] and apply appropriate diagnostic criteria when screening for PH.”

Where Experts Agree

Specialists who were surveyed about screening for PH in patients with adult congenital heart disease concurred that all patients with adult congenital heart disease should be screened. There was, however, no evidence supporting a timeline for such follow-up.1

In using echocardiography for detecting PH, the experts agreed that clinicians should refer patients to a cardiac catheterization laboratory to confirm the diagnosis.1 They also cautioned about using similar parameters to assess PH in patients without congenital heart disease because the presentations differ greatly between patients with and without congenital heart disease.1  

“Even though cardiac catheterization is required for establishing the diagnosis of PH and deciding on further management, echocardiography plays an important role in raising the suspicion of PH and referring patients for further investigations,” said Dr Dimopoulos.

Follow-Up Echocardiography for PAH

For patients who have been diagnosed with PAH, follow-up assessment with echocardiography is essential to assess right ventricular function, one of the primary determinants of outcome.3 In checking the hemodynamics of PH, experts suggested that preload and afterload measures should include PA diastolic pressure, mean right atrial pressure, mean PA pressure, left ventricular filling pressure, pulmonary vascular resistance, and right ventricle stroke volume (right ventricular outflow tract velocity time integral).3

Wright and colleagues identified 20 variables to assess right ventricle function, a major factor in PH outcomes, after reviewing echocardiography follow-up protocols in the literature. The researchers noted that 3-dimensional echocardiography is usually more cost-effective than cardiac magnetic resonance imaging.3

“We were worried about the problems of PA pressure estimation and how much they are the source of attention in follow-up, when the evidence really shows us that it’s the right ventricle that is the main driver of outcome, and now with right ventricular strain we have a relatively simple test to track this,” explained Professor Thomas H. Marwick, MBBS, PhD, MPH, from the Baker-IDI Heart and Diabetes Institute, Melbourne, Victoria, Australia, in an interview with Pulmonology Advisor. “It’s not in guidelines, but in our opinion, [right ventricular] strain should be the primary marker of [right ventricular] function at both baseline and follow-up.”

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Summary & Clinical Applicability

Echocardiography is the recommended method to screen for PAH. In adults with congenital heart disease, however, clinicians need to consider anatomic abnormalities that occur in subtypes of adult congenital heart disease and adapt their screening parameters.

Limitations & Disclosures



  1. Dimopoulos K, Condliffe R, Tulloh RMR, et al. Echocardiographic screening for pulmonary hypertension in congenital heart disease: JACC review topic of the week. J Am Coll Cardiol. 2018;72(22):2778-2788.
  2. Galiè N, Beghetti M, Gatzoulis MA, et al for the Bosentan Randomized Trial of Endothelin Antagonist Therapy-5 (BREATHE-5) Investigators. Bosentan therapy in patients with Eisenmenger syndrome: a multicenter, double-blind, randomized, placebo-controlled study. Circulation. 2006;114(1):48-54.
  3. Wright LM, Dwyer N, Celermajer D, Kritharides L, Marwick TH. Follow-up of pulmonary hypertension with echocardiography. JACC Cardiovasc Imaging. 2016;9(6):733-746.