Mild therapeutic hypothermia (TH) is an established therapy to improve survival and reduce neurologic injury after cardiac arrest. Adult patients with congenital heart disease (ACHD) are at increased risk of sudden cardiac death. The use of TH in this population has not been extensively studied. The aim of this study is to report our institutional experience using this treatment modality in patients with ACHD after cardiac arrest. We performed a retrospective observational study of a cohort of 245 consecutive patients treated with TH after cardiac arrest from 2007 to 2013. Five patients were identified as having complex ACHD with a mean age of 28 years. All were treated with TH according to an institutional protocol utilizing active surface cooling to maintain a core body temperature of 32°C to 34°C for 24 hours after cardiac arrest. Congenital lesions in these 5 patients included anomalous left coronary artery from the pulmonary artery; l -transposition of the great arteries; d -transposition of the great arteries status post atrial switch; unoperated tricuspid atresia, atrial septal defect, and ventricular septal defect with Eisenmenger’s physiology; and surgically corrected atrial septal defect, cleft mitral valve, and subaortic membrane. All 5 patients suffered cardiac arrest due to ventricular arrhythmia and all survived to discharge without significant neurologic impairment. Therapeutic interventions included anomalous left coronary artery from the pulmonary artery ligation, percutaneous coronary intervention, and defibrillator implantation. In conclusion, in 5 patients with ACHD, the use of TH after cardiac arrest resulted in 100% survival to hospital discharge with good neurologic outcome postresuscitation.
Cardiac arrest is a major public health concern associated with significant morbidity and mortality for which mild therapeutic hypothermia (TH) is a recommended therapy. Given their young age, increased susceptibility to sudden cardiac death, and the potential for therapeutic interventions after recovery, TH is an attractive treatment modality in adult patients with congenital heart disease (ACHD) who suffer cardiac arrest. We hypothesize that TH can be used to improve survival and reduce neurologic injury in patients with ACHD postarrest. In this retrospective study, we report a series of 5 patients with complex ACHD who presented with sudden cardiac death and were treated with TH. The aim of this study is to report our institutional experience with the implementation, safety, and efficacy of TH in this unique patient population.
Methods
The study population included 245 consecutive comatose patients after sudden cardiac death who were treated with TH at the Vanderbilt University Medical Center from 2007 to 2013. Patients determined by their treating physician to be suitable for TH were externally cooled to maintain a target body temperature of 32°C to 34°C for 24 hours after return of spontaneous circulation, after which they were passively rewarmed at a rate of 0.25°C per hour. After approval from the institutional review board, data were collected retrospectively including medical history, initial rhythm, time to return of spontaneous circulation, receipt of bystander cardiopulmonary resuscitation, length of intensive care unit and hospital stay, complications, and cerebral performance category (CPC) score at hospital discharge and follow-up. Patients were considered for inclusion in this study if they had a history of moderate or complex congenital heart disease, suffered a primary cardiac arrest, and underwent mild TH postresuscitation.
The primary outcome for this study was CPC at hospital discharge. The CPC score was developed as a measure of central nervous system function after cardiac arrest and is the most commonly used postresuscitation outcome measure for this purpose. A CPC score of 1 indicates normal neurologic function, a CPC score of 2 to 4 indicates progressive degrees of neurologic injury, and a CPC score of 5 indicates death. We defined a CPC score of 1 to 2 as a good neurologic outcome.
Results
Baseline patient characteristics, hospital course, and outcomes are listed in Table 1 . All patients suffered cardiac arrest due to ventricular arrhythmia and successfully completed treatment with mild hypothermia. Ventricular arrhythmia was the initial rhythm in each patient with ACHD compared with 132 of 245 (54%) of the remaining cohort. All patients with ACHD survived to hospital discharge with good neurologic function (CPC score 1 or 2) and to a mean of 14-month follow-up. Furthermore, each adult congenital patient underwent an intervention to address the potential underlying etiology of sudden cardiac death including ligation of anomalous left coronary artery from the pulmonary artery; percutaneous coronary intervention; and defibrillator implantation (4 of 5 patients). In the postdischarge follow-up period, 1 patient underwent successful heart-lung transplantation, whereas another received biventricular mechanical support as a bridge to transplant. The 2 remaining patients are currently awaiting listing for heart transplantation.
| Variable | Patient | ||||
|---|---|---|---|---|---|
| #1 | #2 | #3 | #4 | #5 | |
| Age (years) at arrest | 20 | 23 | 27 | 34 | 38 |
| Sex | Female | Male | Male | Male | Female |
| Type of congenital disease | ALCAPA | L-TGA | D-TGA | ASD, tricuspid atresia, VSD | ASD, subaortic membrane, cleft MV |
| Initial rhythm | VF | VT | VF | VT | VF |
| Time to ROSC (minutes) | 11 | 15 | 20 | 10 | 20 |
| Location of arrest | Out of hospital | Out of hospital | In hospital | Out of hospital | Out of hospital |
| Bystander CPR | Yes | Yes | N/A | Yes | Yes |
| Time to initiation of TH (minutes) | 60 | 52 | 20 | 32 | 35 |
| Time to reach target temperature (minutes) | 120 | 266 | 56 | 180 | 95 |
| Complications during hospitalization | 0 | 0 | Pneumonia | Pneumonia, AF | 0 |
| Length of ICU stay (days) | 2 | 4 | 5 | 4 | 4 |
| CPC at ICU discharge | 1 | 2 | 1 | 1 | 2 |
| Length of hospitalization (days) | 7 | 10 | 37 | 15 | 11 |
| CPC at hospital discharge | 2 | 1 | 1 | 1 | 1 |
| Follow-up interval (months) | 17 | 11 | 14 | 15 | X |
| CPC at follow-up | 1 | 1 | 1 | 1 | X |
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