Table 1.

Characteristics and comorbidities of patients with hereditary hemorrhagic telangiectasia (HHT) undergoing general anesthesia

	Patients (n = 74)
Male sex	30 (40.5)
Body mass index (kg m−2)	26.9 ± 6.9
ASA-PS	3 [2, 4]
Adult patients	65 (87.8)
Age at first surgery (years)	55.4 [20.0, 86.1]
Pediatric patients	9 (12.2)
Age at first surgery (years)	7.2 [0.7, 13.9]
Range at first surgery (years)	0.7–13.9
Curacao score
Definite HHT (Curacao score >2)	59 (79.7)
Suspected HHT (Curacao score = 2)	15 (20.3)
Spontaneous and recurrent epistaxis	67
Multiple mucocutaneous telangiectasias	60
Visceral involvement	67
First-degree relative with HHT	47
Cardiovascular disease	28 (37.8)a
Coronary artery disease	7
Heart failure	17
Left ventricular failure	3
Right ventricular failure	3
High-output failure	11
Valvular disease	4
Atrial fibrillation	12c
Pulmonary disease	51 (68.9)a
Arteriovenous malformation	42
Pulmonary hypertension	11
Home oxygen	2
Pulmonary embolism history	7
Moderate–severe COPD	6
Home oxygen	2
Neurological disease	16 (21.6)a
Arteriovenous malformation	7b
Brain abscess	1
Cerebral hemorrhage	2
Stroke or transient ischemic attack	7
Seizure disorder	2
Encephalopathy	1
Hepatic disease	29 (39.2)a
Arteriovenous malformation	25
Cirrhosis/portal hypertension	4
Gastrointestinal bleed	25 (33.8)
Iron deficiency anemia	48 (64.7)
End-stage kidney disease	2 (2.7)

Data presented at number of patients (percentage), mean ± standard deviation for continuous variables, or median [range] [interquartile range] for nonparametric variables

HHT hereditary hemorrhagic telangiectasia, ASA-PS American Society of Anesthesiologists physical status, COPD chronic obstructive lung disease

^aPatients may have multiple conditions per organ system class

^bSeven patients had arteriovenous malformations of the brain and one also of the thoracic spine

^cAtrial fibrillation was only found in adult patients, of which 5 had congestive heart failure, 4 valvular disease, 3 pulmonary hypertension, and 10 iron deficiency anemia