PMID- 28743113
OWN - NLM
STAT- MEDLINE
DCOM- 20180718
LR  - 20180718
IS  - 1423-0356 (Electronic)
IS  - 0025-7931 (Linking)
VI  - 94
IP  - 3
DP  - 2017
TI  - Pulmonary Hypertension in a Large Cohort with Hereditary Hemorrhagic 
      Telangiectasia.
PG  - 242-250
LID - 10.1159/000458447 [doi]
AB  - BACKGROUND: Hereditary hemorrhagic telangiectasia (HHT) is a vascular disorder 
      characterized by arteriovenous malformations in the brain, liver, and lungs. 
      Pulmonary hypertension (PH) is increasingly recognized as a severe complication 
      of HHT. However, there are no studies describing the prevalence of PH in HHT 
      compared to HHT-negative controls. OBJECTIVE: To assess the estimated prevalence 
      of PH in patients with HHT compared to HHT-negative controls. METHODS: All 
      consecutive subjects screened for HHT with available genetic testing and 
      echocardiography-based peak tricuspid regurgitation velocity (TRV) measurement 
      were included. Increased-probability PH was defined as a TRV >2.8 m/s. RESULTS: 
      In 578 subjects, both echocardiography and genetic testing were available. A 
      reliable TRV was measured in 383 (66.3%), of whom 127 had HHT type 1 (HHT1), 150 
      had HHT type 2 (HHT2), and 106 were HHT-negative controls, with a mean TRV of 2.3 
      +/- 0.4, 2.4 +/- 0.5, and 2.2 +/- 0.3 m/s, respectively (p = 0.008 and p < 0.001 vs. 
      controls). Increased-probability PH was found in 42 subjects (8.7% in HHT1, 18.0% 
      in HHT2, and 3.8% in HHT-negative controls). HHT2 and hepatic arteriovenous 
      malformations (HAVMs) were the most important predictors for 
      increased-probability PH (odds ratio 5.6, p = 0.002, and odds ratio 11.3, p < 
      0.001, respectively). Heritable pulmonary arterial hypertension (HPAH) was 
      diagnosed in 2 patients (0.7%) and only found in HHT2 (1.3%). CONCLUSION: The 
      estimated prevalence of PH is higher in HHT patients compared to HHT-negative 
      controls. This increase is especially present in HHT2 and mainly associated with 
      the presence of HAVMs. HPAH appears to be rare in HHT patients and was only 
      diagnosed in HHT2.
CI  - (c) 2017 S. Karger AG, Basel.
FAU - Vorselaars, Veronique
AU  - Vorselaars V
AD  - Department of Cardiology, St. Antonius Hospital Nieuwegein, Nieuwegein, The 
      Netherlands.
FAU - Velthuis, Sebastiaan
AU  - Velthuis S
FAU - van Gent, Marco
AU  - van Gent M
FAU - Westermann, Cornelis
AU  - Westermann C
FAU - Snijder, Repke
AU  - Snijder R
FAU - Mager, Johannes
AU  - Mager J
FAU - Post, Martijn
AU  - Post M
LA  - eng
PT  - Journal Article
DEP - 20170726
PL  - Switzerland
TA  - Respiration
JT  - Respiration; international review of thoracic diseases
JID - 0137356
SB  - IM
MH  - Adult
MH  - Case-Control Studies
MH  - Female
MH  - Humans
MH  - Hypertension, Pulmonary/epidemiology/*etiology
MH  - Male
MH  - Middle Aged
MH  - Netherlands/epidemiology
MH  - Prevalence
MH  - Telangiectasia, Hereditary Hemorrhagic/*complications/epidemiology
OTO - NOTNLM
OT  - Echocardiography
OT  - Hereditary hemorrhagic telangiectasia
OT  - Heritable pulmonary arterial hypertension
OT  - Pulmonary hypertension
EDAT- 2017/07/26 06:00
MHDA- 2018/07/19 06:00
CRDT- 2017/07/26 06:00
PHST- 2016/06/02 00:00 [received]
PHST- 2016/12/09 00:00 [accepted]
PHST- 2017/07/26 06:00 [pubmed]
PHST- 2018/07/19 06:00 [medline]
PHST- 2017/07/26 06:00 [entrez]
AID - 000458447 [pii]
AID - 10.1159/000458447 [doi]
PST - ppublish
SO  - Respiration. 2017;94(3):242-250. doi: 10.1159/000458447. Epub 2017 Jul 26.