Humera Ahmed1, Jeffrey B Anderson2, Katherine E Bates3, Shobha Natarajan4, Nancy S Ghanayem5, Carole M Lannon2, David W Brown6. 1. Departments of Cardiology, Seattle Children's Hospital, University of Washington, Seattle, WA, USA. 2. Cincinnati Children's Hospital Medical Center, Cincinnati, OH, USA. 3. C.S. Mott Children's Hospital, University of Michigan, Ann Arbor, MI, USA. 4. Children's Hospital of Philadelphia, Philadelphia, PA, USA. 5. Cardiology, Advocate Children's Heart Institute, Oak Lawn, IL, USA. 6. Division of Cardiology, Boston Children's Hospital, 300 Longwood Avenue, Boston, MA, 02115, USA. david.brown@cardio.chboston.org.
Abstract
BACKGROUND: Interstage mortality (IM) remains high for patients with single-ventricle congenital heart disease (SVCHD) in the period between Stage 1 Palliation (S1P) and Glenn operation. We sought to characterize IM. METHODS: This was a descriptive analysis of 2184 patients with SVCHD discharged home after S1P from 60 National Pediatric Cardiology Quality Improvement Collaborative sites between 2008 and 2015. Patients underwent S1P with right ventricle-pulmonary artery conduit (RVPAC), modified Blalock-Taussig-Thomas shunt (BTT), or Hybrid; transplants were excluded. RESULTS: IM occurred in 153 (7%) patients (median gestational age 38 weeks, 54% male, 77% white), at 88 (IQR 60,136) days of life, and 39 (IQR 17,84) days after hospital discharge; 13 (8.6%) occurred ≤ 30 days after S1P. The mortality rate for RVPAC was lower (5.2%; 59/1138) than BTT (9.1%; 65/712) and Hybrid (20.1%; 27/134). More than half of deaths occurred at home (20%) or in the emergency department (33%). The remainder occurred while inpatient at center of S1P (cardiac intensive care unit 36%, inpatient ward 5%) or at a different center (5%). Fussiness and breathing problems were most often cited as harbingers of death; distance to surgical center was the biggest barrier cited to seeking care. Cause of death was unknown in 44% of cases overall; in the subset of patients who underwent post-mortem autopsy, the cause of death remained unknown in 30% of patients, with the most common diagnosis being low cardiac output. CONCLUSIONS: Most IM occurred in the outpatient setting, with non-specific preceding symptoms and unknown cause of death. These data indicate the need for research to identify occult causes of death, including arrhythmia.
BACKGROUND: Interstage mortality (IM) remains high for patients with single-ventricle congenital heart disease (SVCHD) in the period between Stage 1 Palliation (S1P) and Glenn operation. We sought to characterize IM. METHODS: This was a descriptive analysis of 2184 patients with SVCHD discharged home after S1P from 60 National Pediatric Cardiology Quality Improvement Collaborative sites between 2008 and 2015. Patients underwent S1P with right ventricle-pulmonary artery conduit (RVPAC), modified Blalock-Taussig-Thomas shunt (BTT), or Hybrid; transplants were excluded. RESULTS: IM occurred in 153 (7%) patients (median gestational age 38 weeks, 54% male, 77% white), at 88 (IQR 60,136) days of life, and 39 (IQR 17,84) days after hospital discharge; 13 (8.6%) occurred ≤ 30 days after S1P. The mortality rate for RVPAC was lower (5.2%; 59/1138) than BTT (9.1%; 65/712) and Hybrid (20.1%; 27/134). More than half of deaths occurred at home (20%) or in the emergency department (33%). The remainder occurred while inpatient at center of S1P (cardiac intensive care unit 36%, inpatient ward 5%) or at a different center (5%). Fussiness and breathing problems were most often cited as harbingers of death; distance to surgical center was the biggest barrier cited to seeking care. Cause of death was unknown in 44% of cases overall; in the subset of patients who underwent post-mortem autopsy, the cause of death remained unknown in 30% of patients, with the most common diagnosis being low cardiac output. CONCLUSIONS: Most IM occurred in the outpatient setting, with non-specific preceding symptoms and unknown cause of death. These data indicate the need for research to identify occult causes of death, including arrhythmia.
Authors: Jeffrey B Anderson; Robert H Beekman; John D Kugler; Geoffrey L Rosenthal; Kathy J Jenkins; Thomas S Klitzner; Gerard R Martin; Steven R Neish; David W Brown; Colleen Mangeot; Eileen King; Laura E Peterson; Lloyd Provost; Carole Lannon Journal: Circ Cardiovasc Qual Outcomes Date: 2015-06-09
Authors: Humera Ahmed; Jeffrey B Anderson; Katherine E Bates; Craig E Fleishman; Shobha Natarajan; Nancy S Ghanayem; Lynn A Sleeper; Carole M Lannon; David W Brown Journal: J Thorac Cardiovasc Surg Date: 2019-11-14 Impact factor: 5.209
Authors: S P McGuirk; M Griselli; O F Stumper; E M Rumball; P Miller; R Dhillon; J V de Giovanni; J G Wright; D J Barron; W J Brawn Journal: Heart Date: 2005-06-06 Impact factor: 5.994
Authors: N S Ghanayem; G M Hoffman; K A Mussatto; J R Cava; P C Frommelt; N A Rudd; M M Steltzer; S M Bevandic; S S Frisbee; R D B Jaquiss; S B Litwin; J S Tweddell Journal: J Thorac Cardiovasc Surg Date: 2003-11 Impact factor: 5.209
Authors: James S Tweddell; George M Hoffman; Kathleen A Mussatto; Raymond T Fedderly; Stuart Berger; Robert D B Jaquiss; Nancy S Ghanayem; Stephanie J Frisbee; S Bert Litwin Journal: Circulation Date: 2002-09-24 Impact factor: 29.690
Authors: Valerie Bailey; Dorothy M Beke; Jennifer M Snaman; Faraz Alizadeh; Sarah Goldberg; Melissa Smith-Parrish; Kimberlee Gauvreau; Elizabeth D Blume; Katie M Moynihan Journal: JAMA Netw Open Date: 2022-05-02