Pulmonary Artery Dilatation Correlates With the Risk of Unexpected Death in Chronic Arterial or Thromboembolic Pulmonary Hypertension

doi:10.1378/chest.11-2794

Chest

Volume 142, Issue 6, December 2012, Pages 1406-1416

https://doi.org/10.1378/chest.11-2794 Get rights and content

Background

Right ventricular failure does not explain all cases of death in patients with chronic pulmonary hypertension. Searching for alternative explanations, we evaluated the prognostic significance of main pulmonary artery (PA) dilatation in patients with pulmonary arterial hypertension (PAH) or chronic thromboembolic pulmonary hypertension (CTEPH).

Methods

A retrospective outcome analysis was made of 264 patients (aged 46 ± 17 years; women, 69%; PAH, 82%) who underwent both CT scan measurement of the PA and right-sided heart catheterization (mean PA pressure, 57.6 ± 16.5 mm Hg) at initial evaluation.

Results

The diameter of the PA ranged from 28 to 120 mm (mean, 39 ± 8.6 mm; median, 38 mm) and was largest in patients with unrepaired congenital defects (42.6 ± 7.6 mm). Pulmonary pulse pressure (P = .04), lower age (P = .03), and duration of symptoms (P < .001) were independently but weakly related to PA diameter. During follow-up (median, 38 months), 99 patients (37%) died. Of these 99 deaths, 73 (74%) were due to heart failure or comorbidities, and 26 (26%) were unexpected deaths (UE-Ds). PA diameter (hazard ratio [HR], 1.06 per 1 mm; 95% CI, 1.03-1.08), heart rate (HR, 1.30 per 10 beats/min; 95% CI, 1.01-1.66), and systolic pulmonary arterial pressure (HR, 1.02 per 1 mm Hg; 95% CI, 1.01-1.04) were the only independent predictors of UE-D and differed from the usual predictors found in the study group for all-cause mortality. PA diameter ≥ 48 mm had 95% specificity and 39% sensitivity and carried 7.5 times higher risk of UE-D (95% CI, 3.4-16.5; P < .0001) during follow-up.

Conclusions

PA dilatation emerges as an independent risk factor for death unexplained by right ventricular failure or comorbidities in patients with PAH and CTEPH. The possible mechanisms include, but are not limited to, PA compression of the left main coronary artery, PA rupture, or dissection with cardiac tamponade.

Section snippets

Materials and Methods

We retrospectively analyzed the outcome of 300 patients referred to the PH center between 1998 and 2009 who were followed by our team because of the diagnosis of PAH or chronic thromboembolic PH (CTEPH) unsuitable for surgical treatment. The diagnosis was made based on an algorithm including right-sided heart catheterization. The treatment after enrollment followed the best locally available treatment strategy. Two hundred sixteen patients (72%) were treated with targeted therapy, which

Basic Characteristics and Hemodynamic Data

Clinical and hemodynamic data of 264 patients included in the analysis and 36 (12%) excluded from the trial because of unavailable baseline CT scans are presented in Table 1. Most of the 264 patients with available baseline CT scans were initially classified as World Health Organization (WHO) functional class III (n = 147, 55%) or functional class II (n = 105, 40%). They presented with significantly compromised exercise tolerance (mean distance of 6-min walk test, 385 ± 126 m) and elevated

Discussion

RV failure resulting in low cardiac output is the main cause of death in patients with PAH.1, 2, 15 However, with growing experience of referral centers, new potentially life-threatening complications are being recognized. Supraventricular arrhythmias,¹⁶ recurrent haemoptysis,¹⁷ angina due to compression of the main left coronary artery,⁴ and management of PAH in pregnancy and labor¹⁸ exemplify new challenges faced by clinical teams caring for patients with PAH. Moreover, some otherwise

Conclusions

PA dilatation emerges as an independent risk factor for death unexplained by RV failure or comorbidities in patients with PAH or inoperable CTEPH. Possible mechanisms include the consequences of LMCA compression and also PA rupture or dissection with cardiac tamponade. Further studies on the clinical significance of PA dilatation are required to understand the causes of death and, we hope, to improve the outcome of patients with PH.

Acknowledgments

Author contributions: Drs Żyłkowska, Kurzyna, and Torbicki had full access to all of the data in the study and take responsibility for the integrity of the data and the accuracy of data analysis.

Dr Żyłkowska: contributed to conception and design of the study, analysis and interpretation of data, drafting part of the manuscript and revising it critically for important intellectual content, and giving final approval of the manuscript before submission.

Dr Kurzyna: contributed to conception and

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We applied a fully-automatic in-house developed algorithm for segmentation of arteries and veins to determine lung vessel morphology in patients with precapillary PH who underwent right heart catheterization and CTPA between May 2016 and May 2019. Primary endpoint of this retrospective study was the calculation of receiver operating characteristics for identifying low and high mortality risk according to the 3-strata risk assessment model presented in the current guidelines.
We analyzed 73 patients, median age 65 years (interquartile range (IQR): 54-76), female/male ratio 35/38, median mean pulmonary arterial pressure 37 mm Hg (IQR: 30-46), and found significant correlations with important prognostic factors in pulmonary arterial hypertension. N-terminal pro-brain natriuretic peptide, cardiac index, mixed venous oxygen saturation, and 6-minute walking distance were correlated with the ratio of the number of arteries over veins with vessel diameters of 6-10 mm (Spearman correlation coefficients ρ = 0.64, p < 0.001; ρ = −0.60, p < 0.001; ρ = −0.47, p = 0.005; ρ = −0.45, p = 0.001, respectively). This ratio predicted a low- and high-risk score with an area under the curve of 0.73 (95% confidence interval (CI): 0.56-0.90) and 0.86 (95% CI: 0.74-0.97), respectively.
The ratio of the number of arteries over veins with diameters between 6 and 10 mm is significantly correlated with prognostic markers in pulmonary hypertension and predicts low and high mortality risk.
Echocardiographic reference intervals of the dimensions of the main pulmonary artery and the right pulmonary artery: a prospective study in 269 healthy dogs
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No data are available on the echocardiographic reference intervals (RIs) for the main pulmonary artery (MPA) and right pulmonary artery (RPA) dimensions in a large sample of dogs. Therefore, we aimed to describe the echocardiographic RIs of the MPA and RPA dimensions in normal dogs.
Two hundred and sixty nine healthy dogs of different breeds, age and body weight (BW) were prospectively enrolled in this multicenter, observational study. The MPA diameter, RPA maximum diameter (RPAmax), and RPA minimum diameter (RPAmin) were measured from the right parasternal short axis view. Prediction intervals (PIs) for MPA, RPAmax and RPAmin were generated using allometric scales. Reference intervals (RI) of MPA indexed to the ascending aorta (MPA/AO), and RPAmax and RPAmin indexed to the aortic annulus (RPAmax/Aod and RPAmin/Aod), were defined.
A positive linear relationship between MPA, RPAmax, RPAmin and BW was evident after logarithmic transformation (R² = 0.859, R² = 0.787 and R² = 0.725, respectively; P<0.0001). According to allometric scales, the PI for the MPA normalized for BW (MPA_N) was between 5.50 and 8.07, the PI for the RPAmax normalized for BW (RPAmax_N) was between 3.23 and 5.62, while the PI for the RPAmin normalized for BW (RPAmin_N) was between 1.62 and 3.30. The median MPA/AO was 0.92 (RI, 0.78–1.01), the median RPAmax/Aod was 0.70 (RI, 0.53–0.98) and the median RPAmin/Aod was 0.40 (RI, 0.29–0.61).
The reported RIs of the MPA and RPA dimensions in normal dogs could increase the diagnostic accuracy of transthoracic echocardiography in the identification of pulmonary artery enlargement in dogs.
Association Between the Degree of Severity of Pulmonary Hypertension With the Presence of Pulmonary Artery Aneurysm: A Brief Updated Review for Clinicians
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Pulmonary hypertension (PH) is defined as an increase in mean pulmonary arterial pressure (mPAP) ≥20 mm Hg at rest as assessed by right cardiac catheterization. It has a median survival nowadays of 6 years, compared to 2.8 years in the 1980s. A pulmonary artery aneurysm (PAA) is the focal dilation of a blood vessel involving all 3 layers of the vessel wall; they have a diameter greater than 4 cm measured in the trunk of the pulmonary artery. PAAs can be classified into proximal (or central) and peripheral. The clinical manifestations of PAA are primarily nonspecific, and most patients remain undiagnosed, even those with large PAA, due to its silent course; however, clinical manifestations occur unless when there are complications such as bronchial or tracheal compression (leading to cough and dyspnea), dissection, or rupture (leading to hemoptysis). PAH is observed in 66% of patients with PAA. PA dissections are usually associated with PAH; 80% of dissections occur in the main pulmonary trunk. Although there is no clear guideline for the best treatment of PAA, surgery is indicated in patients with a pulmonary trunk aneurysm >5.5 cm. It has been observed that patients in the PAH group associated with congenital heart disease tend to develop PAA more commonly. Those with PAH associated with connective tissue disease have a smaller diameter of PA dilation. This report presents a comprehensive review of PAA, discussing critical aspects of the clinical and imaging diagnosis, hemodynamics, and treatment. A comprehensive updated literature review is included; we believe this article will interest cardiopulmonologists.
Risk of pulmonary artery dissection in adults with congenital heart disease
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There are limited data about the risk of pulmonary artery (PA) dissection in adults with congenital heart disease (CHD), and the purpose of this study was to estimate the incidence of PA dissection in this population.
Retrospective cohort study of adults with CHD that underwent cross-sectional imaging (2003–2020). PA aneurysm was defined as main or branch PA diameter > 40 mm or > 30 mm respectively, and severe PA aneurysm was defined as main or branch PA diameter > 50 mm.
Of 1, 673 patients (41 ± 10 years; male 58%), 493 (24%), 286 (19%), and 306 (20%) had aneurysms of the main, right, and left PA respectively, while 66 (4%) had severe PA aneurysm. During a median follow-up of 8.2 (interquartile range 3.7–10.3) years, there was one PA dissection in a patient with Eisenmenger syndrome, thus the incidence of PA dissection was 14 per 100,000 patient-years.
Of 779 females, 163 had one or more pregnancies during follow-up, and 41 (25%) of these patients had known PA aneurysm at the time of conception. There was no PA dissection during pregnancy. Of the 163 patients, 91 (56%) had cross-sectional imaging before and after pregnancy, there was no significant difference in PA dimension before versus after pregnancy (main PA 35 ± 5 versus 36 ± 4 mm, p = 0.6; right PA 21 ± 3 versus 33 ± 4 mm, p = 0.1; and left PA 23 ± 4 versus 22 ± 4 mm, p = 0.4).
The risk of PA dissection was extremely low even in patients with severe PA aneurysm, or in patients with PA aneurysm that became pregnant. Collectively, these data suggest a benign natural history for patients without severe pulmonary hypertension and encourage and a conservative approach in managing patients with PA aneurysm.
Larger pulmonary artery to ascending aorta ratios are associated with decreased survival of patients undergoing pulmonary endarterectomy
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Citation Excerpt :
Various other studies found correlations between PA diameter and outcome variables in different patient cohorts. The extent of PA dilatation was found to be associated with more severe forms of PH defined by an mPAP >30 mm Hg in patients with heart failure with preserved ejection fraction,10 as well as a significantly higher rate of unexpected deaths in patients with PAH or CTEPH.27 Moreover, PA diameters were shown to be significantly larger in patients with severe aortic stenosis and PH compared with those without signs for PH.28 In terms of outcome after BPA for inoperable CTEPH, larger PA diameters were associated with a higher amount of mild complications postinterventionally.29
The ratio of pulmonary artery (PA) and ascending aorta (AA) diameters has recently been shown to be a useful indicator for disease severity and predictor of outcome in patients with pulmonary hypertension and heart failure. This study aimed at evaluating the applicability of this ratio for perioperative risk assessment of patients with chronic thromboembolic pulmonary hypertension undergoing pulmonary endarterectomy.
In this retrospective cohort study on 149 patients undergoing pulmonary endarterectomy between 2013 and 2020, the preoperative PA to AA ratio was analyzed on axial computed tomography. Variables of pulmonary hemodynamic status were assessed during preoperative right heart catheterization and postoperative Swan-Ganz catheter measurements. Perioperative survival was analyzed by Kaplan-Meier method and log-rank tests.
Preoperative computed tomography measurements showed a median AA diameter of 31 mm (range, 19-47 mm), and a median PA diameter of 36 mm (range, 25-55 mm). The calculated median PA to AA ratio was 1.13 (range, 0.79-1.80). PA to AA ratio correlated positively with PA pressure (systolic, r = 0.352 [P < .001]; diastolic, r = 0.406 [P < .001]; mean, r = 0.318 [P < .001]) and inversely with age (r = −0.484 [P < .001]). Univariable Cox regression analysis identified PA diameter (P = .008) as a preoperative parameter predictive of survival. There was a significant difference (log-rank P = .037) in 30-day survival probability for patients with lower PA to AA ratios (<1.136; survival probability, 97.4%) compared with patients with higher ratios (>1.136; survival probability, 88.9%).
PA to AA ratio shows a correlation with other variables associated with pulmonary hypertension. In addition, patients with higher PA to AA ratios have lower survival probabilities after PEA. Further analysis of PA to AA ratio on the selection of chronic thromboembolic pulmonary hypertension for different treatment modalities—pulmonary endarterectomy, medical therapy, and or balloon pulmonary angioplasty—is warranted.
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In addition to the pericardium, other sites of extension include into the lung parenchyma, mediastinum, and pleural space, also resulting in sudden death. An increased risk for PAD and unexpected death has been reported for pulmonary artery diameters measuring greater than 4.8 cm [10]. Most cases of PAD have been diagnosed at autopsy.
Pulmonary arterial dissection (PAD) is a rare and often lethal complication of chronic pulmonary arterial hypertension (PAH), which may occurs in patients with idiopathic pulmonary arterial hypertension (IPAH) and potentially in those with connective tissue disorders. While rare, sudden death often occurs secondary to acute cardiac tamponade, as the pulmonary artery dissects into the pericardium; this diagnosis is often made postmortem. Nevertheless, with the proliferation of multidetector computed tomography (MDCT) as a diagnostic test, patients may be identified very early after symptom onset, prompting rapid intervention with decreased morbidity and mortality. We report a case of IPAH complicated by pulmonary artery aneurysm (PAA) and PAD, diagnosed by CT pulmonary angiogram (CTPA), and treated with bilateral lung transplantation, pulmonic valve replacement, and re-anastomosis of the donor main PA to a pulmonary valve conduit.

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Drs Żyłkowska and Kurzyna contributed equally to this article.

Funding/Support: The authors have reported to CHEST that no funding was received for this study.

Reproduction of this article is prohibited without written permission from the American College of Chest Physicians. See online for more details.

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Chest

Original ResearchPulmonary Vascular DiseasePulmonary Artery Dilatation Correlates With the Risk of Unexpected Death in Chronic Arterial or Thromboembolic Pulmonary Hypertension

Background

Methods

Results

Conclusions

Section snippets

Materials and Methods

Basic Characteristics and Hemodynamic Data

Discussion

Conclusions

Acknowledgments

Am J Med

Pathology

J Thorac Cardiovasc Surg

Am Heart J

Chest

Chest

J Am Coll Cardiol

Cardiovasc Revasc Med

Gastroenterology

J Am Coll Cardiol

Characteristics and prognosis of patients with decompensated right ventricular failure during the course of pulmonary hypertension

Kardiol Pol

Prognostic factors of acute heart failure in patients with pulmonary arterial hypertension

Eur Respir J

Survival in pulmonary arterial hypertension: a reappraisal of the NIH risk stratification equation

Eur Respir J

Fatal dissection of the pulmonary artery in pulmonary arterial hypertension

Eur Respir Rev

Pulmonary artery dissection: an emerging cardiovascular complication in surviving patients with chronic pulmonary hypertension

Heart

Original Research
Pulmonary Vascular Disease
Pulmonary Artery Dilatation Correlates With the Risk of Unexpected Death in Chronic Arterial or Thromboembolic Pulmonary Hypertension