scholarly journals A Retrospective Review of Patients with Severe World Health Organization Group 3 Pulmonary Hypertension Evaluated for Lung Transplant: A Single Center Experience

Author(s):  
R. Marron ◽  
S. Eswarakrishnan ◽  
V. Kim ◽  
F.C. Cordova ◽  
S.E. Weaver
Circulation ◽  
2020 ◽  
Vol 142 (Suppl_3) ◽  
Author(s):  
Martin Marak ◽  
Danielle Tatum ◽  
Denzil Moraes

Introduction: The World Health Organization (WHO) categorizes pulmonary hypertension (PHTN) into 5 groups. Group II classification denotes PHTN secondary to left-heart disease and accounts for nearly 75% of all cases. However, there is limited data regarding the effect of PHTN Group II status on outcomes in the perioperative setting. Hypothesis: PHTN WHO Group II is an independent risk factor for adverse cardiopulmonary events in the perioperative setting. Methods: Retrospective review of patients who underwent intra-abdominal surgery between January 2014 - August 2019 and had previously obtained an echocardiogram. PHTN Group II was defined as estimated pulmonary artery pressure (EPAP) > 30mmHg on echocardiogram . Other forms of PHTN were excluded. Major adverse cardiovascular events (MACE) were defined as heart failure exacerbation, arrhythmia, myocardial infarction, 30 day readmission, and death. Surgical risk was categorized as low (laparoscopic) or intermediate (open). Results: By echocardiogram findings, 65 of the 178 (36.3%) patients included were Group II PHTN. Between surgical risk classes, Group II PHTN was older (mean age 73.7 years v 60.5; P< 0.01), had more comorbidities including systolic (9.70% v 21.5%, P=0.03) and diastolic (22.1% v 34.5%, P < 0.01) heart failure, and were more likely to have a MACE ( 6.2% v 43.1%, P < 0.01). PHTN Group II patients with intermediate-risk surgeries demonstrated significantly more MACE than control (11.2% v 43.7%, P <0.01) without significant difference in comorbidities. Conclusions: Group II PHTN is an independent risk factor for MACE in patients undergoing intermediate risk surgery compared to non-PHTN counterparts. Additional studies involving severity of pulmonary hypertension may provide further insight into risk stratification.


2019 ◽  
Vol 9 (4) ◽  
pp. 204589401989667
Author(s):  
Kentaro Ejiri ◽  
Satoshi Akagi ◽  
Kazufumi Nakamura ◽  
Naofumi Amioka ◽  
Keishi Ichikawa ◽  
...  

Hereditary haemorrhagic telangiectasia or Rendu-Osler-Weber syndrome is a systemic vascular disease with autosomal dominant inheritance, mucocutaneous telangiectasia, and repeated nasal bleeding due to vascular abnormalities. Hereditary haemorrhagic telangiectasia may occasionally lead to complications, including arteriovenous malformations and pulmonary hypertension. We present a case of a 52-year-old female patient with hereditary haemorrhagic telangiectasia who was referred to our hospital for treatment of pulmonary hypertension. She had been diagnosed with hereditary haemorrhagic telangiectasia during adolescence and was being followed up. Six months prior to presentation, she had undergone coil embolization for pulmonary haemorrhage due to pulmonary arteriovenous malformations. She was in World Health Organization functional class IV, with a mean of pulmonary arterial pressure of 38 mmHg, a pulmonary capillary wedge pressure of 10 mmHg, and a right atrial pressure of 22 mmHg. A contrast-enhanced computed tomography angiography showed large arteriovenous malformations in the liver. Right heart catheterization revealed an increase in oxygen saturation in the inferior vena cava between the supra- and infra-hepatic veins, low pulmonary vascular resistance, and high right atrial pressure. Hence, she was diagnosed with hereditary haemorrhagic telangiectasia with pulmonary hypertension due to major arteriovenous shunt resulting from arteriovenous malformations in the liver. Therefore, we considered liver transplantation as an essential treatment option. She underwent cadaveric liver transplantation after a year resulting in dramatic haemodynamic improvement to World Health Organization functional class I. Liver transplantation is a promising treatment in patients with hereditary haemorrhagic telangiectasia and pulmonary hypertension resulting from arteriovenous shunt caused by arteriovenous malformations in the liver.


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