Chronic thromboembolic pulmonary hypertension in pediatric patients.
OBJECTIVE
Chronic thromboembolic pulmonary hypertension is a rare form of pulmonary hypertension that can lead to progressive right heart failure and death. Pulmonary thromboendarterectomy surgery is the treatment of choice resulting in significant improvements in functional status, cardiopulmonary hemodynamics, and survival. This study reports the largest case series of pediatric patients with chronic thromboembolic pulmonary hypertension who underwent pulmonary thromboendarterectomy surgery at one institution.


METHODS
The University of California, San Diego, chronic thromboembolic pulmonary hypertension database identified patients 18 years or younger at the time of pulmonary thromboendarterectomy surgery (n = 17). Medical charts were reviewed for hemodynamics, thromboembolic risk factors, and postoperative outcomes.


RESULTS
Pulmonary thromboendarterectomy surgery in pediatric patients resulted in improved functional status and significantly improved cardiopulmonary hemodynamics: mean arterial pressure decreased from 45.5 mm Hg ± 20.7 to 27.3 ± 13.0 mm Hg (P = .00073), pulmonary vascular resistance decreased from 929 ± dynes · s · cm(-5) to 299 ± 307 dynes · s · cm(-5) (P = .0012), and cardiac output improved from 3.8 ± 1.1 L/min to 5.6 ± 1.6 L/min (P = .0061). There were no deaths during surgery or 30 days after surgery, and long-term survival (5+ years) was achieved in 87.5%. As compared to adults with chronic thromboembolic pulmonary hypertension, there was a higher rate of rethrombosis in pediatric patients (38% vs 1%-4%).


CONCLUSIONS
This study demonstrates that pulmonary thromboendarterectomy surgery in pediatric patients with chronic thromboembolic pulmonary hypertension is well tolerated with improved postoperative hemodynamics, functional status, minimal postoperative complications, and low perioperative mortality, similar to that reported for adults with chronic thromboembolic pulmonary hypertension, with the notable exception being a higher rate of rethrombosis in pediatric patients.
Madani
Michael M
MM
Department of Cardiothoracic Surgery, University of California San Diego, San Diego, Calif 92103-8892, USA. mmadani@ucsd.edu
Wittine
Lara M
LM
Auger
William R
WR
Fedullo
Peter F
PF
Kerr
Kim M
KM
Kim
Nick H
NH
Test
Victor J
VJ
Kriett
Jolene M
JM
Jamieson
Stuart W
SW
D000293:Adolescent; D000959:Antihypertensive Agents / Q000627:therapeutic use; D002140:California; D002648:Child; D002908:Chronic Disease; D004691:Endarterectomy / Q000009:adverse effects / Q000401:mortality; D005260:Female; D006439:Hemodynamics; D006801:Humans; D006976:Hypertension, Pulmonary / Q000209:etiology / Q000401:mortality / Q000503:physiopathology / Q000601:surgery*; D008297:Male; D020127:Recovery of Function; D012008:Recurrence; D012189:Retrospective Studies; D018570:Risk Assessment; D012307:Risk Factors; D012720:Severity of Illness Index; D015996:Survival Rate; D013923:Thromboembolism / Q000150:complications / Q000401:mortality / Q000503:physiopathology / Q000601:surgery*; D013997:Time Factors; D016896:Treatment Outcome
D000959:Antihypertensive Agents