What is the clinical evidence from this study?

Study design: Cohort. Population: Adult congenital heart disease (CHD) (n=38430). Intervention: Pulmonary hypertension vs. No pulmonary hypertension. Primary outcome: All-cause mortality (HR 2.69, 95% CI 2.41-2.99).

What does this research mean for the field?

A diagnosis of pulmonary hypertension in adults with congenital heart disease is associated with a more than two-fold higher risk of all-cause mortality and a three-fold higher risk of morbid complications. Novelty: ClaimNovelty.INCREMENTAL. Consensus alignment: ConsensusAlignment.NEUTRAL.

September 18, 2026Journal of the American College of CardiologyOpen Access

Diagnosis of Pulmonary Hypertension in the Congenital Heart Disease Adult Population

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Key result

Pulmonary hypertension in adults with congenital heart disease is linked to ~2.7-fold higher all-cause mortality.

HR 2.69
95% CI 2.41-2.99
n=38,430

Why the study?

Population-based studies assessing the impact of pulmonary hypertension on survival and functional capacity in adults with congenital heart disease are uncommon.

Population

38,430 adults with congenital heart disease in 2005

Comparison

Adults with congenital heart disease with vs without pulmonary hypertension diagnosis

Design

Retrospective longitudinal cohort study

Follow-up

23 years (1983 to 2005)

Authors

BLB. LoweAuckland City Hospital JTJudith TherrienPediatric / Congenital Cardiology RIRaluca Ionescu‐IttuStatistics Canada

Discussion

Member takes

Implication

Retrospective cohort study reveals increased mortality and morbidity in adults with congenital heart disease and pulmonary hypertension, highlighting substantial clinical and healthcare burdens.

Key Points

To assess the impact of pulmonary hypertension on mortality, morbidity, and health services utilization in an adult congenital heart disease population.
Retrospective longitudinal cohort study of adults with congenital heart disease (N=38,430 alive in 2005) followed for 23 years from 1983 to 2005.
Mortality, morbidity, and health services utilization were evaluated between matched subcohorts with and without pulmonary hypertension using multivariate Cox and Poisson regression models.
Pulmonary hypertension was present in 5.8% (n=2,212) of patients and was associated with a greater than two-fold increase in all-cause mortality (HR: 2.69; 95% CI: 2.41 to 2.99).
Morbid complications, including heart failure and arrhythmia, occurred with a three-fold higher risk in patients with pulmonary hypertension (HR: 3.01; 95% CI: 2.80 to 3.22).
Health services utilization was elevated, including non-diagnostic cardiac catheterizations (rate ratio: 5.04; 95% CI: 4.27 to 5.93) and coronary or intensive care hospitalizations (rate ratio: 5.03; 95% CI: 4.86 to 5.20).

Study Design

Type

Cohort (n=38,430)

PICO

Population

38,430 adults with congenital heart disease, followed for up to 23 years to assess the impact of pulmonary hypertension on mortality, morbidity, and health services utilization.

Exposure / Comparator

Pulmonary hypertension vs No pulmonary hypertension

Primary Outcome

All-cause mortality — HR 2.69 (2.41-2.99)

Main Result

Hazard Ratio: 2.69 (95% CI 2.41–2.99)

Cite This Study

Lowe et al. (2011) conducted a cohort in Adult congenital heart disease (CHD) (n=38,430). Pulmonary hypertension vs. No pulmonary hypertension was evaluated on All-cause mortality (HR 2.69, 95% CI 2.41-2.99). A diagnosis of pulmonary hypertension in adults with congenital heart disease was associated with a more than 2-fold higher risk of all-cause mortality (HR 2.69; 95% CI 2.41-2.99).

synapsesocial.com/papers/6aad01580c7bbc3e61ee9e50 https://doi.org/10.1016/j.jacc.2011.03.033

Topics

Heart failure HFrEF treatment Heart failure hospitalization Echocardiography Congenital heart disease adults

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Implication

Retrospective cohort study reveals increased mortality and morbidity in adults with congenital heart disease and pulmonary hypertension, highlighting substantial clinical and healthcare burdens.

Diagnosis of Pulmonary Hypertension in the Congenital Heart Disease Adult Population

Discussion

Member takes

Implication

Key Points

Study Design

PICO

Main Result

Cite This Study

Topics

Diagnosis of Pulmonary Hypertension in the Congenital Heart Disease Adult Population

Discussion

Member takes

Implication

Key Points

Study Design

PICO

Main Result

Cite This Study

Also Consider

Topics

Also Consider