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Think Outside the Heart: Noncardiac Complications and Considerations in the Adult Congenital Patient

Disclosure: None
Pub Date: Monday, Oct. 9, 2017
Author: Leigh Christopher Reardon, MD, FACC
Affiliation: Ahmanson/UCLA Adult Congenital Heart Disease Center

Citation

Lui GK, Saidi A, Bhatt AB, Burchill LJ, Deen JF, Earing MG, Gewitz M, Ginns J, Kay JD, Kim YY, Kovacs AH, Krieger EV, Wu FM, Yoo S-J; on behalf of the American Heart Association Adult Congenital Heart Disease Committee of the Council on Clinical Cardiology and Council on Cardiovascular Disease in the Young; Council on Cardiovascular Radiology and Intervention; and Council on Quality of Care and Outcomes Research. Diagnosis and management of noncardiac complications in adults with congenital heart disease: a scientific statement from the American Heart Association [published online ahead of print October 9, 2017]. Circulation. doi: 10.1161/CIR.0000000000000535.

Article Text

Tackling the big issues facing the adult congenital heart disease (ACHD) population continues to gain traction in a field that has now come of age. In the past two years, we have seen AHA scientific statements on topics ranging from heart failure and mechanical support to the management of pregnancy in complex congenital heart disease.1-3 We also have the implementation of new training guidelines, an American Board of Internal Medicine (ABIM) board examination (co-sponsored by the American Board of Pediatrics), and ACHD care center accreditation through the Adult Congenital Heart Association (ACHA).

Still, some of the most complex issues responsible for significant morbidity and mortality in the ACHD patient lie outside of the heart. Lui and colleagues diligently undertake this endeavor with a comprehensive review of noncardiac complications in ACHD patients.4 For the experienced ACHD practitioner, each section conjures up thoughts of patients that have had such complications and leaves one with greater comprehension and strategies to address these issues. For the trainee in adult or pediatric cardiology or for those not specifically trained or specialized in ACHD, the document serves as a road map to understanding, diagnosing and treating the noncardiac challenges for ACHD patients.

The very fact that we are now facing these long-term extra-cardiac challenges in ACHD is a testament to the profound improvements in surgery, imaging, medical therapy and catheter based interventions that have expanded the population of congenital heart disease (CHD) patients and resulted in adult survival estimates of greater than 90%.5 The most recent statistics estimate 1 in 150 adults have some form of CHD. The number of adults with CHD has surpassed the number of children – estimated at 1,400,000 adults compared to 1,000,000 children in 2010 in the United States.6 Nearly one-third of these patients have severe forms of CHD. Thus, we have a generation of patients who are at risk of developing chronic multisystem disease in adulthood secondary to their hemodynamic derangements as well as the normal processes of aging.

ACHD patients frequently interface with the medical system for their noncardiac illnesses well after they have attained significant morbidity. Routine surveillance is only gaining widespread acceptance particularly for screening of renal, pulmonary and hepatic disease.7 This in the face of almost 50% of young adults with impaired renal function highlighting the intricate connection of the cardiorenal system.8 Furthermore, early detection of liver disease in this population of patients who suffer from hemodynamic stresses (venous congestion, ischemic injury) and non-hemodynamic factors such as hepatitis C infection are paramount. Particularly concerning is the recognition of increasing numbers of patients with oncologic diagnoses.9  Patients with CHD have risk factors that start with in-utero hemodynamic derangements which then compound and evolve from various surgical palliations and associated cardiac bypass exposures.

Additionally, many a patient have been psychosocially disabled by one’s relationship with his/her own chronic disease that may result in a life of inactivity, poor dietary habits and limited emotional well-being.10 While these issues and the resultant obesity, hypertension, lipid disorders and insulin resistance – to name a few – are pervasive in our society, they are magnified in the ACHD population. A prime example is the large number of patients with unrecognized and untreated sleep apnea – a condition worsened by the prevalence of restrictive and obstructive lung disease secondary to scarring from open chest operations.11 The presence of sleep apnea can have a profound impact on oxygenation and ventilation, development of arrhythmias, and detrimentally affect the hemodynamics of various palliations, most notably patients with a cavopulmonary or Fontan connection.

For many ACHD patients, the most hazardous endeavor is undergoing elective non-cardiac surgery in a setting that is ill equipped to deal with their comorbidities. Many patients will under report their functional limitations, therefore, providers may mistakenly perceive that younger and seemingly well-functioning patients as having near normal cardiopulmonary reserve. Not surprisingly, up to 40% of adverse events in ACHD patients undergoing noncardiac surgery result from inadequate risk assessment in this setting.12

Lui et al. provide caution and guidance for these and many common challenges in ACHD patients and urge an interdisciplinary approach to thinking outside the heart.  The safety, longevity, productivity and well-being of our patients depend on it.

References

  1. Stout KK, Broberg CS, Book WM, Cecchin F, Chen JM, Dimopoulos K, Everitt MD, Gatzoulis M, Harris L, Hsu DT, Kuvin JT, Law Y, Martin CM, Murphy AM, Ross HJ, Singh G, Spray TL; on behalf of the American Heart Association Council on Clinical Cardiology, Council on Functional Genomics and Translational Biology, and Council on Cardiovascular Radiology and Imaging. Chronic heart failure in congenital heart disease: a scientific statement from the American Heart Association [published online ahead of print January 19, 2016]. Circulation. doi: 10.1161/CIR.0000000000000352.
  2. Ross HJ, Law Y, Book WM, Broberg CS, Burchill L, Cecchin F, Chen JM, Delgado D, Dimopoulos K, Everitt MD, Gatzoulis M, Harris L, Hsu DT, Kuvin JT, Martin CM, Murphy AM, Singh G, Spray TL, Stout KK; on behalf of the American Heart Association Adults With Congenital Heart Disease Committee of the Council on Clinical Cardiology and Council on Cardiovascular Disease in the Young, the Council on Cardiovascular Radiology and Intervention, and the Council on Functional Genomics and Translational Biology. Transplantation and mechanical circulatory support in congenital heart disease: a scientific statement from the American Heart Association [published online ahead of print January 21, 2016]. Circulation. doi: 10.1161/CIR.0000000000000353.
  3. Canobbio MM, Warnes CA, Aboulhosn J, Connolly HM, Khanna A, Koos BJ, Mital S, Rose C, Silversides C, Stout K; on behalf of the American Heart Association Council on Cardiovascular and Stroke Nursing; Council on Clinical Cardiology; Council on Cardiovascular Disease in the Young; Council on Functional Genomics and Translational Biology; and Council on Quality of Care and Outcomes Research. Management of pregnancy in patients with complex congenital heart disease: a scientific statement for healthcare professionals from the American Heart Association [published online ahead of print January 12, 2017]. Circulation. doi: 10.1161/CIR.0000000000000458.
  4. Lui GK, Saidi A, Bhatt AB, Burchill LJ, Deen JF, Earing MG, Gewitz M, Ginns J, Kay JD, Kim YY, Kovacs AH, Krieger EV, Wu FM, Yoo S-J; on behalf of the American Heart Association Adult Congenital Heart Disease Committee of the Council on Clinical Cardiology and Council on Cardiovascular Disease in the Young; Council on Cardiovascular Radiology and Intervention; and Council on Quality of Care and Outcomes Research. Diagnosis and management of noncardiac complications in adults with congenital heart disease: a scientific statement from the American Heart Association [published online ahead of print October 9, 2017]. Circulation. doi: 10.1161/CIR.0000000000000535.
  5. Gurvitz M, Burns KM, Brindis R, et al., Emerging Research Directions in Adult Congenital Heart Disease. J Am Coll Cardiol.2016;67: 1956-64.
  6. Gilboa SM, Devine OJ, Kucik JE, et al., Congenital Heart Defects in the United States: Estimating the Magnitude of the Affected Population in 2010. Circulation. 2016;134: 101-109.
  7. Ford RM, Book W and Spivey JR. Liver disease related to the heart. Transplantation reviews (Orlando, Fla). 2015;29:33-7.
  8. Raissadati A, Nieminen H, Haukka J, Sairanen H and Jokinen E. Late Causes of Death After Pediatric Cardiac Surgery: A 60-Year Population-Based Study. J Am Coll Cardiol. 2016;68:487-98.
  9. Dimopoulos K, Diller GP, Koltsida E, Pijuan-Domenech A, Papadopoulou SA, Babu-Narayan SV, Salukhe TV, Piepoli MF, Poole-Wilson PA, Best N, Francis DP and Gatzoulis MA. Prevalence, predictors, and prognostic value of renal dysfunction in adults with congenital heart disease. Circulation. 2008;117:2320-8.
  10. Deng LX, Khan AM, Drajpuch D, Fuller S, Ludmir J, Mascio CE, Partington SL, Qadeer A, Tobin L, Kovacs AH and Kim YY. Prevalence and Correlates of Post-traumatic Stress Disorder in Adults With Congenital Heart Disease. Am J Cardiol. 2016;117:853-7.
  11. Watson NF, Bushnell T, Jones TK and Stout K. A novel method for the evaluation and treatment of obstructive sleep apnea in four adults with complex congenital heart disease and Fontan repairs. Sleep Breath. 2009;13:421-4.
  12. Maxwell BG, Posner KL, Wong JK, Oakes DA, Kelly NE, Domino KB and Ramamoorthy C. Factors contributing to adverse perioperative events in adults with congenital heart disease: a structured analysis of cases from the closed claims project. Congenital Heart Disease. 2015;10:21-9.

-- The opinions expressed in this commentary are not necessarily those of the editors or of the American Heart Association --