PAH Caused by Undiagnosed Systemic Sclerosis in Case Study - Pulmonary Hypertension News

PAH Caused by Undiagnosed Systemic Sclerosis in Case Study - Pulmonary Hypertension News
CTEPH Severity Assessed by Dual Energy CT Parameters and Hemodynamics - Pulmonology Advisor
Pulmonary Artery Distensibility Effective Diagnostic Tool for Pulmonary Hypertension Related to Left Heart Disease - The Cardiology Advisor

Posted: 29 Jul 2020 09:00 AM PDT

A case report found a patient's pulmonary arterial hypertension (PAH) was caused by an undiagnosed case of the autoimmune disorder systemic sclerosis (SSc), highlighting the need for early and thorough diagnostic workups, researchers say.

The 77-year-old woman's PAH was also worsened by other conditions, including lung disease, sleep apnea, heart failure, and chronic anemia, which made the diagnosis difficult.

With multiple possible causes for PAH, researchers recommend that attention be paid to undiagnosed autoimmune disorders that may play a role in the disease's development.

"Development of severe pulmonary hypertension and [lung disease] leading to diagnosis of SSc in this case is unusual," the researchers wrote in the article "Multifactorial Pulmonary Hypertension in Systemic Sclerosis," which was published in the Cureus Journal of Medical Science.

PAH is a chronic and progressive disease characterized by high blood pressure in the pulmonary arteries — the blood vessels that supply blood from the heart to the lungs.

The disease can have many potential causes, including heart abnormalities, viral infections, liver disease, connective tissue disorders such as scleroderma and lupus, and the use of certain medications, or it can be inherited. PAH without an easily identifiable cause is called idiopathic PAH.

When multiple possible causes occur at the same time, diagnosis and treatment can be challenging, as illustrated by the case of the 77-year-old female patient in the study reported by researchers at the University of Illinois College of Medicine.

The patient complained of worsening shortness of breath upon physical exertion for the past nine months, which interfered with her daily activities.

She had a medical history of genetic-related anemia (low red blood cells) and a type of inflammatory bowel disease known as Crohn's disease, and was being treated for both conditions. She reported no history of blood clots, or heart or liver disease, and no family history of connective tissue disorders.

An initial examination found low blood oxygen levels and abnormal rattling sounds in the lungs. The patient was thus given oxygen supplementation.

Blood tests confirmed the presence of antibodies associated with autoimmune disease, which led to a diagnosis of SSc — an autoimmune disease characterized by the buildup of scar tissue in the skin and several internal organs, including the arteries.

Functional and imaging tests showed signs of lung disease. The patient was also diagnosed and treated for sleep apnea, in which pauses in breathing occur during sleep.

Over the next few months, her shortness of breath during physical activity worsened, which was confirmed by a decline in lung function. A sonogram of the heart (echocardiogram) showed high blood pressure in the pulmonary arteries.

The patient was then diagnosed with PAH in a setting of SSc along with lung disease, sleep apnea, heart failure, and chronic anemia.

She declined rapidly over the next three months and was treated for possible pneumonia. She continued to show low blood oxygen levels, and her pulmonary blood pressure had increased.

"With continued increment in oxygen requirement, [lung function tests] and CT findings were out of proportion to the degree of pulmonary hypertension," the team wrote.

The patient started treatment with increasing doses of the blood pressure medication nifedipine (sold under several brand names).

Following a meeting with specialists in lung disease, cardiology, and rheumatology, she was started on a triple combination of PAH therapies which included the blood vessel dilators Uptravi (selexipag) and Opsumit (macitentan), both marketed by Actelion, along with Adcirca (tadalafil, marketed by United Therapeutics).

She also continued treatment with nifedipine, immunosuppressant steroids, and mycophenolate to treat SSc, as well as torsemide to treat fluid overload due to heart failure and high blood pressure.

After six months, her pulmonary blood pressure decreased, which was consistent with mild improvements. Her treatment regimen continued to be optimized.

"Despite evident causes, due consideration should be given to undiagnosed autoimmune disorders playing a role in causing PAH," the researchers wrote, noting that "management in such cases remains a multidisciplinary approach."

Author Details

Steve holds a PhD in Biochemistry from the Faculty of Medicine at the University of Toronto, Canada. He worked as a medical scientist for 18 years, within both industry and academia, where his research focused on the discovery of new medicines to treat inflammatory disorders and infectious diseases. Steve recently stepped away from the lab and into science communications, where he's helping make medical science information more accessible for everyone.

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Patrícia holds her PhD in Medical Microbiology and Infectious Diseases from the Leiden University Medical Center in Leiden, The Netherlands. She has studied Applied Biology at Universidade do Minho and was a postdoctoral research fellow at Instituto de Medicina Molecular in Lisbon, Portugal. Her work has been focused on molecular genetic traits of infectious agents such as viruses and parasites.

CTEPH Severity Assessed by Dual Energy CT Parameters and Hemodynamics - Pulmonology Advisor

Posted: 30 Jul 2020 08:30 AM PDT

In patients with chronic thromboembolic pulmonary hypertension (CTEPH), lung perfused blood volume (PBV) and mean pulmonary artery (PA) enhancement could be indicators of hemodynamics, according the results of a retrospective analysis published in the Journal of Computer Assisted Tomography.

Investigators sought to evaluate the link between dual-energy computed tomography (CT) quantitative parameters and hemodynamics in patients with CTEPH. Between April 2014 and July 2017, a total of 58 consecutive patients who had undergone dual-energy CT for a detailed examination or follow-up of their CTEPH received treatment at Nagoya University Hospital in Japan. A CTEPH diagnosis was confirmed with the use of ventilation/perfusion scintigraphy, right heart catheterization (RHC), and pulmonary arteriography.

Of the 58 consecutive patients, 6 were excluded from the study for a variety of reasons, leaving a total of 52 remaining patients to be assessed retrospectively. Of these 52 patients, 20 were men and 32 were women. The median participant age was 65.5 years. Dual-energy CT and RHC were performed within 6 months of each other. The participants' lung PBV and mean PA enhancement, which were measured at the pulmonary parenchymal phase, were compared with hemodynamics.

Correlations were demonstrated between dual-energy CT parameters and body weight with RHC hemodynamics. Mean lung PBV was significantly correlated with mean pulmonary arterial pressure (mPAP; Spearman rank correlation coefficient [r_s]=0.47; 95% CI, 0.23-0.66; P <.001), systolic ventricular pressure (r_s=0.44; 95% CI, 0.19-0.63; P =.001), right arterial pressure (r_s=0.32; 95% CI, 0.05-0.54; P =.022), and pulmonary vascular resistance (r_s=0.31; 95% CI, 0.04-0.53; P =.027)

In addition, whole lung PBV was significantly correlated with mPAP (P =.010); however the correlation coefficient was lower than that for lung PBV calculated when excluding the upper right zone. Furthermore, PA enhancement was significantly associated with cardiac index (P <.001) and with pulmonary vascular resistance (P <.001). The area under the curve (AUC) was 0.86 for lung PBV to predict mPAP >50 mm Hg. The AUC was also 0.86 for PA enhancement to predict pulmonary vascular resistance >1000 dyne·s/cm⁵.

The investigators concluded that the study findings showed the ability of the quantitative values of lung PBV and PA enhancement to identify patients with severe CTEPH. Thus, lung PBV and PA enhancement could be used as indicators of hemodynamics, with noninvasive quantitative dual-energy CT parameters helping to guide the management of patients with CTEPH.

Reference

Tsutsumi Y, Iwano S, Okumura N, et al. Assessment of severity in chronic thromboembolic pulmonary hypertension by quantitative parameters of dual-energy computed tomography. J Comput Assist Tomogr. 2020;44(4):578-585.

Topics:

Pulmonary Hypertension

Pulmonary Artery Distensibility Effective Diagnostic Tool for Pulmonary Hypertension Related to Left Heart Disease - The Cardiology Advisor

Posted: 17 Jul 2020 12:00 AM PDT

Measurements of pulmonary artery (PA) distensibility were more effective to detect pulmonary hypertension (PH) due to left heart disease (LHD) than measurements of PA dilatation, in patients with severe heart failure with reduced ejection fraction (HFrEF), aortic stenosis, or primary mitral regurgitation, according to study findings published in European Radiology.

In this prospective, diagnostic and prognostic study, 100 patients (mean age, 63±17 years; 79% men) who were considered for heart transplant due to HFrEF, for transcatheter aortic valve implantation due to aortic stenosis, or for surgery due to primary mitral regurgitation at a single center in Belgium, were recruited between 2015 and 2019. All patients underwent right heart catheterization, ungated computed tomography (CT), electrocardiograph (ECG)-gated CT, and echocardiography.

The patients with vs without PH differed significantly in heart rate (76±13 vs 65±11, respectively; P <.001), rates of atrial fibrillation (32% vs 11%, respectively; P <.001), New York Heart Association functional class III or IV (41% vs 20%, respectively; P <.001), HFrEF (59% vs 41%, respectively; P<.001), aortic stenosis (65% vs 35%, respectively; P <.001), and mitral regurgitation (27% vs 73%, respectively; P <.001).

PA distensibility <18% was found to detect PH-LHD with 96% sensitivity, 73% specificity, an area under the receiving operator curve (AUC) of 0.92, which was significantly larger than the AUCs for tricuspid regurgitation gradient (0.83), or for the ratio of PA diameter to aorta diameter (0.82).

PA distensibility was found to correlate with the patient factors of age (r, -0.35; P <.001), heart rate (r, -0.40; P <.001), mean pulmonary artery pressure (r, -0.72; P <.001), pulmonary artery wedge pressure (r, -0.65; P <.001), pulmonary vascular resistance (r, -0.59; P <.001), right PA minimal cross-sectional area (r, -0.59; P <.001), right PA maximal cross-sectional area (r, -0.42; P <.001), cardiac index (r, 0.38; P <.001), and atrial fibrillation (r, -0.27; P =.006) in a univariate analysis. Detection of PA distensibility was reproducible both within (r, 0.96; P <.001) and between (r, 0.89; P <.001) observers.

Predictors for PA distensibility was independently associated, in a multivariate analysis, with mean PA pressure (ß, -0.41; P <.001), minimal right PA area (ß, -0.008; P =.019), and age (ß, -0.07; P =.019).

Limitations of this study include the fact that CT and right heart catheterization were conducted during separate procedures. In addition, CT was performed while the patient held their breath after a deep inspiration and PA pressure was recorded after a long expiration. These factors may have introduced some variation.

"[I]n patients with severe HFrEF, aortic stenosis, or mitral regurgitation, PA distensibility in ECG-gated CT can detect PH-LHD better than the parameters reflecting PA dilatation in ungated CT or TR gradient in echocardiography," concluded the study authors.

Reference

Colin G C, Verlynde G, Pouleur A C, et al. Pulmonary hypertension due to left heart disease: diagnostic value of pulmonary artery distensibility. [Published online June 16, 2020] Eur Radiol. doi:10.1007/s00330-020-06959-7

Topics:

Heart Failure Pulmonary Hypertension Pulmonary Vasculature

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