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Anesthesiology:
doi: 10.1097/ALN.0b013e3182a56e00
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Adult Congenital Heart Disease Patients Undergoing Noncardiac Surgery and the Role of Anesthesiologists as Perioperative Physicians

Cannesson, Maxime M.D., Ph.D.*; Earing, Michael M.D.

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“This article will be of significant value to anesthesiologists, cardiologists, and surgeons who do not routinely take care of these patients.”
In this issue of ANESTHESIOLOGY, Maxwell et al.1 presented an article on the perioperative outcomes of major noncardiac surgery in adults with congenital heart disease. This important study, based on the analysis of the Nationwide Inpatient Sample database, is the first to demonstrate that patients with adult congenital heart disease (ACHD) undergoing noncardiac surgery experienced increased perioperative morbidity and mortality and greater postoperative length of stay and hospital charges compared with a well-matched comparison cohort. This may not come as a surprise to members of the healthcare team who routinely take care of ACHD patients, but it is the first time that such information is clearly reported. This article will be of significant value to anesthesiologists, cardiologists, and surgeons who do not routinely take care of these patients. It will help our community to understand the severity of illness of this population, and the specific care they should receive in the perioperative period.
The management of children with congenital heart diseases (9 in 1,000 live births)2 has dramatically improved during the past 30 yr, and more than 90% of children born with a congenital heart disease survive to adulthood.3 As a consequence, it was recently reported that more adults than children live with a congenital heart disease in the United States today.4 Considering that most of these adult patients will, sooner or later, undergo noncardiac surgery or become pregnant, it is of major importance for any healthcare provider involved in the perioperative period to understand the severity of these patients and to organize, ahead of time, a specific management for this population.
Practically speaking, there are three main informations to take into account when managing ACHD patients for noncardiac surgery such as: (1) the timing of the surgery (scheduled vs. urgent or emergent surgery), (2) the center where the surgery is performed and the expertise available, and (3) the pathology. Although there are no formal guidelines regarding the perioperative management of these patients to date, it is now widely accepted that anesthesiologists and cardiologists with significant expertise with ACHD should manage patients with moderate or severe congenital heart disease presenting for noncardiac surgery (particularly those with poor functional class, pulmonary hypertension, congestive heart failure, and cyanosis).5 At the very least, cardiologists and anesthesiologists with specific expertise in ACHD should evaluate these patients before any final decision to proceed or not with the surgery.5 If the surgery is an emergency, and if there is no anesthesiologist or cardiologist experienced with ACHD available, it is of major importance to consider some key elements regarding the management of these patients.6 The most common long-term complications of ACHD are classified into cardiac and noncardiac complications, and the anesthesiologist should remember that ACHD must always be considered as a systemic disease. The cardiac complications include ventricular failure, severe pulmonary hypertension, arrhythmia and conduction defects, residual shunts, and valvular lesions. These cardiac complications must be evaluated and anticipated before the procedure. The noncardiac complications include erythrocytosis (associated with an increased risk of bleeding that should be anticipated), somatic abnormalities (including facial dysmorphism which may be associated with difficult airway management), central nervous abnormalities (such as seizure disorders), restrictive and obstructive lung disease, renal dysfunction, and liver dysfunction. In addition, the American Heart Association guidelines clearly state that the following defects present an increased risk of postoperative bacterial endocarditis and should receive antibiotic prophylaxis before surgery: unrepaired cyanotic congenital heart disease, including palliative shunts and conduits; completely repaired congenital heart defects with prosthetic material during the first 6 months after the procedure; repaired congenital heart disease with residual defects at the site or adjacent to the site of a prosthetic patch or prosthetic device; and repaired congenital heart disease where a bioprosthetic or mechanical valve was placed.7 During the procedure, anesthesiologists must remember that arterial pressure and SpO2 cannot be measured on the ipsilateral side of a systemic-to-pulmonary shunt and that the insertion of a central venous access has to be done with caution. Given many of these patients will have had multiple previous procedures and may have had previous arterial and venous lines, it is extremely important to understand the anatomy and impact of their previous surgical repairs. In case of right-to-left or left-to-right shunt, it is important to understand how to control systemic and pulmonary flows in order to decrease shunting.6 Finally, in normal conditions, patients with a Fontan circulation should maintain an arterial saturation above 90%. Arterial saturation below 90% in these patients is abnormal and should lead to further evaluation.
The success of anesthesiology as a specialty in the future will depend on our involvement in perioperative medicine and on our ability to improve patient safety in the perioperative period. The subgroup of ACHD patients undergoing noncardiac surgery exemplifies this challenge, which is also a unique opportunity for our specialty. Improvement in these patients’ outcome will only be achievable through a team-based approach. It will require the development of specific clinical pathways identifying patients in the very early phase of their hospitalization and bringing expertise at the bedside in a systematic way. We still need large clinical studies or quality improvement processes to better understand how these patients should be managed. In the meantime, anesthesiologists should be aware of the severity of this population and of the increased risk of morbidity and mortality associated with this condition. Anytime such a patient presents for noncardiac surgery, the primary anesthesiologist should initiate a multidisciplinary discussion including surgeons, primary care physicians, cardiologists, and cardiac anesthesiologists.
The study by Maxwell et al. presented in this issue of ANESTHESIOLOGY is a landmark article that exemplifies how management of patients with ACHD for noncardiac surgery by anesthesiologists can become the cornerstone of perioperative care.
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References

1. Maxwell BG, Wong JK, Kin C, Lobato RL. Perioperative outcomes of major noncardiac surgery in adults with congenital heart disease. Anesthesiology. 2013;119:762–9

2. Perloff JK, Warnes CA. Challenges posed by adults with repaired congenital heart disease. Circulation. 2001;103:2637–43

3. Warnes CA, Liberthson R, Danielson GK, Dore A, Harris L, Hoffman JI, Somerville J, Williams RG, Webb GD. Task force 1: The changing profile of congenital heart disease in adult life. J Am Coll Cardiol. 2001;37:1170–5

4. Marelli AJ, Mackie AS, Ionescu-Ittu R, Rahme E, Pilote L. Congenital heart disease in the general population: Changing prevalence and age distribution. Circulation. 2007;115:163–72

5. Landzberg MJ, Murphy DJ Jr, Davidson WR Jr, Jarcho JA, Krumholz HM, Mayer JE Jr, Mee RB, Sahn DJ, Van Hare GF, Webb GD, Williams RG. Task force 4: Organization of delivery systems for adults with congenital heart disease. J Am Coll Cardiol. 2001;37:1187–93

6. Cannesson M, Earing MG, Collange V, Kersten JR. Anesthesia for noncardiac surgery in adults with congenital heart disease. Anesthesiology. 2009;111:432–40

7. Wilson W, Taubert KA, Gewitz M, Lockhart PB, Baddour LM, Levison M, Bolger A, Cabell CH, Takahashi M, Baltimore RS, Newburger JW, Strom BL, Tani LY, Gerber M, Bonow RO, Pallasch T, Shulman ST, Rowley AH, Burns JC, Ferrieri P, Gardner T, Goff D, Durack DT. Prevention of infective endocarditis: Guidelines from the American Heart Association: A guideline from the American Heart Association Rheumatic Fever, Endocarditis, and Kawasaki Disease Committee, Council on Cardiovascular Disease in the Young, and the Council on Clinical Cardiology, Council on Cardiovascular Surgery and Anesthesia, and the Quality of Care and Outcomes Research Interdisciplinary Working Group. Circulation. 2007;116:1736–54

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