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The review by Jensen et al1 addresses an important need for guidance in anticoagulation management for adults with congenital heart disease (ACHD). The complex interactions of acquired and genetic thrombophilia risk factors have yielded a wide and treacherous playing field that has dissuaded many colleagues from creating formal treatment policies. This excellent set of recommendations by Jensen et al is a welcome first step towards overcoming the challenges posed by anticoagulating adults with congenital heart disease (CHD).
Moderate or complex malformations in ACHD have increased in prevalence by more than 50% in the past decade. These individuals embody the enormous time-related technical advances in diagnostic imaging, CHD surgery, intensive care and interventional cardiac catheterisation now at our disposal. Many may have had long-term palliation with arterial or venous shunts, while others have received anatomical or physiological repair and have late residua and sequelae related to their original anatomy or surgical intervention.
Haemostasis and its regulation in moderate and complex CHD, particularly those associated with cyanosis, is frequently altered, and often profoundly so. Congenital or acquired genetic risk factors that promote thrombosis may co-exist with the CHD. Not surprisingly, thrombin generation, platelet activation and fibrin deposition, even in the absence of detectable thrombosis, is frequently increased pointing to a hypercoagulable state.2
The authors of this review highlight several potential contributors to thrombophilia, including venous stasis, relative iron deficiency, and low oxygen saturation. Surgical sequelae that may further contribute range from localised stenoses causing flow turbulence and endothelial disruption, aneurysm formation precipitating stasis, pulmonary arterial hypertension inducing disordered endothelial function, to massive volume loaded and dysfunctional ventricular chambers. Though these phenomena are known to contribute to thrombophilia, very little is known about their relative contributions and cross-talk within the same individual.
The authors of this review propose that routine use …
Contributors GRV was engaged in the design and writing of the manuscript; JF was involved in the research of data contained within the manuscript, writing of paper and editing final document; JP contributed to writing of the paper, expert input into haemostasis pathophysiology and editing of the final document.
Competing interests None.
Provenance and peer review Commissioned; internally peer reviewed.