PMID- 20412491
OWN - NLM
STAT- MEDLINE
DCOM- 20100720
LR  - 20110505
IS  - 1747-0803 (Electronic)
IS  - 1747-079X (Linking)
VI  - 5
IP  - 2
DP  - 2010 Mar-Apr
TI  - Discontinuous pulmonary arteries do not preclude good Fontan outcomes.
PG  - 168-73
LID - 10.1111/j.1747-0803.2009.00334.x [doi]
AB  - OBJECTIVE: Discontinuous pulmonary arteries are believed to portend poor outcomes 
      for a single ventricle palliation leading to Fontan's operation. This is a single 
      institutional review of patients with single ventricle and discontinuous 
      pulmonary arteries who underwent pulmonary artery centralization as part of 
      staged surgical palliation. DESIGN: The study is a retrospective case series. 
      PATIENTS: From November 1997 to December 2005, 12 centralization procedures were 
      performed on 12 single ventricle patients with discontinuous pulmonary arteries. 
      The diagnoses at surgery were as follows: heterotaxy 67%, pulmonary atresia 75%, 
      a single morphologic right ventricle 58%, a single morphologic left ventricle 
      33%, and functional single ventricle with atrial situs inversus 8%. OUTCOME 
      MEASURES: The outcome was assessed by hospital survival, actuarial survival, and 
      New York Heart Association (NYHA) classification at follow-up. RESULTS: The 
      overall actuarial survival following centralization is 100% (95% confidence 
      interval = 0.698 to 1). Seventy-five percent of the patients have undergone a 
      Fontan procedure. Median McGoon ratio pre-Fontan = 1.65 (range: 1-2.1). Median 
      follow-up after Fontan = 4.4 years (range: 1.2-9 years). Overall actuarial 
      survival following Fontan is 100% (95% confidence interval = 0.428-0.911). 
      Following the Fontan, there have been no thromboembolic complications, 
      protein-losing enteropathy, nor Fontan takedowns. One hundred percent of the 
      Fontan patients are NYHA class I. CONCLUSIONS: This experience indicates that a 
      resuscitative strategy for discontinuous pulmonary arteries can result in good 
      outcomes after the Fontan procedure. The presence of discontinuous pulmonary 
      arteries in patients with single ventricle physiology should not preclude a 
      management strategy with the goal of Fontan candidacy.
FAU - Randall, Alyssa
AU  - Randall A
AD  - Department of Pediatrics, Baylor College of Medicine, Texas Children's Hospital, 
      Houston, TX 77030, USA. randall@bcm.edu
FAU - Carberry, Kathleen
AU  - Carberry K
FAU - Fraser, Charles D Jr
AU  - Fraser CD Jr
LA  - eng
PT  - Journal Article
PL  - United States
TA  - Congenit Heart Dis
JT  - Congenital heart disease
JID - 101256510
SB  - IM
MH  - Child
MH  - Child, Preschool
MH  - Female
MH  - *Fontan Procedure/adverse effects
MH  - Heart Defects, Congenital/physiopathology/*surgery
MH  - Heart Ventricles/abnormalities
MH  - Hemodynamics
MH  - Humans
MH  - Infant
MH  - Infant, Newborn
MH  - Male
MH  - Palliative Care
MH  - Postoperative Complications
MH  - Pulmonary Artery/*abnormalities
MH  - Retreatment
MH  - Treatment Outcome
EDAT- 2010/04/24 06:00
MHDA- 2010/07/21 06:00
CRDT- 2010/04/24 06:00
PHST- 2010/04/24 06:00 [entrez]
PHST- 2010/04/24 06:00 [pubmed]
PHST- 2010/07/21 06:00 [medline]
AID - CHD334 [pii]
AID - 10.1111/j.1747-0803.2009.00334.x [doi]
PST - ppublish
SO  - Congenit Heart Dis. 2010 Mar-Apr;5(2):168-73. doi: 
      10.1111/j.1747-0803.2009.00334.x.