PMID- 31084317
OWN - NLM
STAT- MEDLINE
DCOM- 20190902
LR  - 20211204
IS  - 2150-136X (Electronic)
IS  - 2150-1351 (Linking)
VI  - 10
IP  - 3
DP  - 2019 May
TI  - Early Experience With Sacubitril/Valsartan in Adult Patients With Congenital 
      Heart Disease.
PG  - 292-295
LID - 10.1177/2150135119825599 [doi]
AB  - BACKGROUND: Heart failure (HF) is the leading cause of hospitalizations and death 
      in patients with adult congenital heart disease (ACHD). Sacubitril/valsartan is a 
      new agent in the treatment of HF, but its effects have not been assessed in ACHD. 
      METHODS: We retrospectively studied all 15 patients with ACHD at our center who 
      were prescribed sacubitril/valsartan between June 2017 and June 2018. We assessed 
      baseline characteristics and clinical and laboratory changes after initiation of 
      sacubitril/valsartan. Adverse events, including renal function, medication 
      intolerance, and worsening HF were documented. RESULTS: The median age was 53.2 
      (27.6-83.6) years, with a median follow-up duration of 69 (8-419) days. At 
      baseline, all patients had refractory HF despite guideline-directed medical 
      therapy, with ten (67%) patients as New York Heart Association (NYHA) class II, 
      and five (33%) patients NYHA class III. The medication was discontinued in one 
      (7%) patient secondary to worsening kidney function. No patients reported 
      clinical deterioration; four NYHA class III patients with complex CHD, pulmonary 
      hypertension, and cyanosis reported significant improvement to NYHA class II. 
      Baseline creatinine was 1.1 (0.9-1.7) and two weeks after starting 
      sacubitril/valsartan it was 1.3 (0.8-2.5, P = .22). CONCLUSIONS: 
      Sacubitril/valsartan seems to be well tolerated in patients with ACHD who present 
      with refractory HF symptoms. Patients with complex CHD associated with cyanosis 
      and pulmonary hypertension could benefit the most, but larger studies are needed 
      to assess the safety as well as the effectiveness of sacubitril/valsartan in this 
      patient population.
FAU - Lluri, Gentian
AU  - Lluri G
AD  - 1 Ahmanson/UCLA Adult Congenital Heart Disease Center, Division of Cardiology, 
      Department of Medicine, David Geffen School of Medicine at UCLA, Los Angeles, CA, 
      USA.
FAU - Lin, Jeannette
AU  - Lin J
AD  - 1 Ahmanson/UCLA Adult Congenital Heart Disease Center, Division of Cardiology, 
      Department of Medicine, David Geffen School of Medicine at UCLA, Los Angeles, CA, 
      USA.
FAU - Reardon, Leigh
AU  - Reardon L
AD  - 1 Ahmanson/UCLA Adult Congenital Heart Disease Center, Division of Cardiology, 
      Department of Medicine, David Geffen School of Medicine at UCLA, Los Angeles, CA, 
      USA.
FAU - Miner, Pamela
AU  - Miner P
AD  - 1 Ahmanson/UCLA Adult Congenital Heart Disease Center, Division of Cardiology, 
      Department of Medicine, David Geffen School of Medicine at UCLA, Los Angeles, CA, 
      USA.
FAU - Whalen, Katrina
AU  - Whalen K
AD  - 1 Ahmanson/UCLA Adult Congenital Heart Disease Center, Division of Cardiology, 
      Department of Medicine, David Geffen School of Medicine at UCLA, Los Angeles, CA, 
      USA.
FAU - Aboulhosn, Jamil
AU  - Aboulhosn J
AD  - 1 Ahmanson/UCLA Adult Congenital Heart Disease Center, Division of Cardiology, 
      Department of Medicine, David Geffen School of Medicine at UCLA, Los Angeles, CA, 
      USA.
LA  - eng
PT  - Journal Article
PL  - United States
TA  - World J Pediatr Congenit Heart Surg
JT  - World journal for pediatric & congenital heart surgery
JID - 101518415
RN  - 0 (Aminobutyrates)
RN  - 0 (Angiotensin II Type 1 Receptor Blockers)
RN  - 0 (Angiotensin Receptor Antagonists)
RN  - 0 (Biphenyl Compounds)
RN  - 0 (Drug Combinations)
RN  - 0 (Tetrazoles)
RN  - 80M03YXJ7I (Valsartan)
RN  - EC 3.4.24.11 (Neprilysin)
RN  - WB8FT61183 (sacubitril and valsartan sodium hydrate drug combination)
SB  - IM
MH  - Adult
MH  - Aged
MH  - Aged, 80 and over
MH  - Aminobutyrates/*therapeutic use
MH  - Angiotensin II Type 1 Receptor Blockers/therapeutic use
MH  - Angiotensin Receptor Antagonists/therapeutic use
MH  - Biphenyl Compounds
MH  - Drug Combinations
MH  - Female
MH  - Follow-Up Studies
MH  - Heart Defects, Congenital/complications/*drug therapy
MH  - Heart Failure/diagnosis/*drug therapy/etiology
MH  - Humans
MH  - Male
MH  - Middle Aged
MH  - Neprilysin
MH  - Retrospective Studies
MH  - Stroke Volume/*physiology
MH  - Tetrazoles/*therapeutic use
MH  - Time Factors
MH  - Treatment Outcome
MH  - Valsartan/*therapeutic use
OTO - NOTNLM
OT  - adult congenital heart disease
OT  - heart failure
OT  - sacubitril/valsartan
EDAT- 2019/05/16 06:00
MHDA- 2019/09/03 06:00
CRDT- 2019/05/16 06:00
PHST- 2019/05/16 06:00 [entrez]
PHST- 2019/05/16 06:00 [pubmed]
PHST- 2019/09/03 06:00 [medline]
AID - 10.1177/2150135119825599 [doi]
PST - ppublish
SO  - World J Pediatr Congenit Heart Surg. 2019 May;10(3):292-295. doi: 
      10.1177/2150135119825599.