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Updated: Aug 20, 2025

Implantation of Total Artificial Heart in Congenital Heart Disease
Published on: July 18, 2014
Outcomes of malignancy in adults with congenital heart disease: a single center experience
Prashanth Venkatesh1, Kimberly L Yan2, Katia Bravo-Jaimes3
1Guerin Congenital Heart Program, Department of Cardiology, Cedars-Sinai Medical Center, 127 S. San Vicente Blvd, Suite A3600, Los Angeles, CA, 90048, USA. prv1405@gmail.com.
Insights
Adult congenital heart disease (ACHD) patients with cancer face significant mortality, primarily from cancer itself. Cardiovascular events are common, but cardiotoxicity rarely forces cancer treatment changes.
Area of Science:
- Cardiology
- Oncology
- Adult Congenital Heart Disease
Background:
- Malignancy is a leading cause of death in adult congenital heart disease (ACHD) patients.
- Limited data exists on cardiovascular and cancer outcomes in this population.
Purpose of the Study:
- To investigate cardiovascular and cancer-related outcomes in patients with ACHD and malignancy.
- To identify predictors of mortality and major adverse cardiovascular and cerebrovascular events (MACCE).
Main Methods:
- Retrospective single-center cohort study of ACHD patients with cancer.
- Primary outcome: all-cause mortality.
- Secondary outcomes: MACCE, cardiotoxicity, and cancer therapy modifications.
Main Results:
- 24% of 68 ACHD patients with cancer died over 5 years, mostly from cancer (69%).
- Predictors of mortality included baseline arrhythmia, diuretic therapy, and advanced cancer stage.
- 59% experienced MACCE, independently predicted by diuretic use; 14% had cardiotoxicity.
Conclusions:
- ACHD patients with cancer experience substantial cancer-related mortality.
- High rates of MACCE were observed, but cardiotoxicity rarely necessitated cancer therapy changes.
Background:
Malignancy is known to be a major cause of death in adult congenital heart disease (ACHD). However, data regarding cardiovascular and cancer-related outcomes in ACHD are lacking.
Methods:
We conducted a retrospective single-center cohort study comprising patients with ACHD and malignancy. The primary outcome was all-cause mortality. Key secondary outcomes included major adverse cardiovascular and cerebrovascular events (MACCE), cardiotoxicity events and consequent cancer therapy modifications.
Results:
Sixty-eight patients with ACHD and a history of cancer were included in the study. 82% of patients had moderate or great ACHD anatomic complexity. Over a median follow-up of 5 years after cancer diagnosis, 16 (24%) patients died, with 69% of deaths being due to cancer. Univariate predictors of mortality were baseline arrhythmia (OR 3.82, 95% CI 1.15-12.67, p = 0.028), baseline diuretic therapy (OR 3.54, 95% CI 1.04-12.04, p = 0.044) and advanced cancer stage at diagnosis (OR 2.37, 95% CI 1.32-4.25, p = 0.004). MACCE occurred in 40 (59%) patients and was independently predicted by baseline diuretic requirement (OR 9.91, 95% CI 1.12-87.85, p = 0.039). A 14% incidence of cardiotoxicity was seen; 3 patients needed modification and 1 patient needed temporary interruption of cancer therapy for 2 weeks.
Conclusions:
Considerable mortality occurred in this cohort of patients with ACHD and cancer; most deaths were cancer-related. A high rate of MACCE was observed, yet rates of obligatory modification of cancer therapy due to cardiotoxicity were low.
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