scholarly journals Reversible Pulmonary Hypertension Associated with Whipple’s Disease

2012 ◽  
Vol 2012 ◽  
pp. 1-2
Author(s):  
A. Villa ◽  
G. Nucera ◽  
A. Kostihova ◽  
A. Mazzola ◽  
P. Marino

We describe a case of Whipple’s disease with pulmonary hypertension in a 72-year-old woman in whom the pulmonary hypertension resolved completely after antibiotic therapy. She was admitted to study with a 2-months history of weight loss, diarrhoea, abdominal pain, asthenia, inappetence, and fever. She did not have dyspnoea or respiratory symptoms. A casual echocardiogram showed a pulmonary artery systolic pressure of 95 mmHg. Forty days after starting antibiotic therapy, an echocardiogram showed a complete normalisation of right ventricular involvement. Whipple’s disease is a rare and multisystemic disorder in which pulmonary involvement is not a well-known finding. Although Whipple’s disease is not generally considered as a possible cause of pulmonary hypertension, such awareness is important because it may be potentially resolved with antibiotic therapy.

Blood ◽  
2013 ◽  
Vol 122 (21) ◽  
pp. 2950-2950
Author(s):  
John Allen Steuter ◽  
Philip Bierman ◽  
R. Gregory Bociek ◽  
Martin Bast

Abstract Anthracycline-based chemotherapy is widely used in a variety of regimens for Hodgkin’s Lymphoma (HL) and Non-Hodgkin’s Lymphoma (NHL). Unfortunately this agent is associated with cardiotoxicity, especially in larger cumulative doses. Doxorubicin cardiotoxicity is characterized by a dose-dependent decline in mitochondrial oxidative phosphorylation. Reactive oxygen species, generated by the interaction of doxorubicin with iron, can then damage the myocytes causing myofibrillar loss, cytoplasmic vacuolization, and apoptosis. Current NCCN guidelines for HL and NHL state that left ventricle function is recommended in patients who will receive such chemotherapy. Subsequent evaluations are recommended at a cumulative dose of doxorubicin of at least 300 mg/m² and periodically thereafter during the course of therapy. Despite these recommendations, there is little literature about the usefulness of this approach, the rate of abnormal findings, and whether routine testing changes management. A list of patients with HL or NHL treated with anthracycline-based chemotherapy from the University of Nebraska Medical Center from August 2004-May 2012 was obtained from our Lymphoma Registry. Baseline characteristics of age, disease diagnosis, gender, and prior history of cardiac diagnosis were collected. Prior cardiac diagnosis specified as patients with a past history of atrial fibrillation/flutter, supraventricular tachycardia, heart block requiring pacemaker implantation, coronary artery disease, valvular disease, pulmonary hypertension, or cardiomyopathy. Charts were reviewed for pre-chemotherapy evaluation of left ventricular function and the method of evaluation (echocardiogram, nuclear, cardiac MRI were included). Additionally, post treatment evaluation of cardiac function was evaluated for any change. The individual therapy for each patient was reviewed to determine if findings from the cardiac evaluation modified treatment regimens. A left ventricular function less than 50% was considered abnormal. Echocardiogram findings of moderate to severe valvular disease, diastolic dysfunction (grade 1-3), and moderate or severe pulmonary hypertension (moderate = pulmonary artery systolic pressure of 45-60, severe = pulmonary artery systolic pressure of > 60) were collected. We identified 309 patients from the UNMC lymphoma database. Of these 219 (71%) had an echocardiogram performed prior to therapy and documented in the patient record. Their mean age was 54.2 years of age with 53% of them being male and 47% female. 188 of the 219 (86%) had no prior cardiac diagnosis as defined previously. From this group 22 of the 188 (10%) had a pre-chemotherapy echocardiogram that demonstrated one of the following: moderate-severe valvular disease, diastolic dysfunction, moderate-severe pulmonary hypertension or abnormal EF (<50%). However, none of these findings altered the chemotherapy regimen for the 22 patients. 31 of the 219 patients with echocardiograms carried a prior cardiac diagnosis. 4 of the 31 had an alteration in their chemotherapy regimen as a result. Post therapy echocardiograms showed no change in cardiac function. 5 of the 219 patients were diagnosed with adriamycin induced cardiomyopathy following treatment. All 5 patients had no prior cardiac history and pre-chemotherapy echocardiograms were normal. These finding suggest that current methods of evaluating cardiac function prior to chemotherapy and risk stratifying patients are inadequate and do not alter patient outcomes. Disclosures: No relevant conflicts of interest to declare.


2016 ◽  
Vol 30 (1) ◽  
pp. 37-42
Author(s):  
Mostashirul Haque ◽  
Tunaggina Afrin Khan ◽  
Md Rasul Amin ◽  
Montasin Rezwan ◽  
Md Rashidul Hasan ◽  
...  

Sarcoidosis is a well known systemic disorder that invariably involves lungs. However, pulmonary hypertension and cor-pulmanle are not common due to pulmonary involvement of sarcoidosis. We report a case of pulmonary hypertension leading to corpulmonale due to sarcoidosis. An elderly female suffering from hypertension & sarcoidosis developed complications and arrived at the diagnosis by correlating various investigations. X-ray chest (P/A) showed cardiomegaly (right ventricular type), reticulo-nodular shadow involving both mid and lower zone; characteristic high resolution CT (HRCT) scan appearances include reticulonodular opacities; pulmonary function test showed restrictive type of defect; ECG showed right ventricular hypertrophy with strain pattern; in echocardiogram there were right ventricular hypertrophy (RVH) with pulmonary artery systolic pressure (PASP) of 63 mmHg indicating severe pulmonary arterial hypertension (PH); along with significantly elevated B-type natriuretic peptide(BNP) level.Bangladesh Heart Journal 2015; 30(1) : 37-42


2011 ◽  
Vol 18 (5) ◽  
pp. e70-e72 ◽  
Author(s):  
Salah Najm ◽  
Joud Hajjar ◽  
Robert P Nelson ◽  
Ramana S Moorthy ◽  
Karen M Wolf ◽  
...  

Pulmonary hypertension (PH) associated with Whipple’s disease (WD-PH) is extremely rare, and the underlying pathophysiological processes are incompletely understood. Alterations in hemodynamics can be severe, with right ventricular (RV) dysfunction being common. A case involving a 23-year-old man with WD-PH who exhibited a dramatic vasodilator response during right heart catheterization despite severely altered pulmonary hemodynamics and concomitant RV dysfunction is reported. While the patient’s symptoms responded poorly to treatment with nifedipine and sildenafil, significant improvement in dyspnea, RV dysfunction and pulmonary pressures were noted following antibiotic therapy. The present report highlights that despite severely elevated pulmonary artery pressures and RV dysfunction in WD-PH patients, a highly significant vasodilator response and dramatic improvement with antibiotic therapy may be observed. Furthermore, the case highlights the phenomenon of PH in the setting of inflammation, suggesting that adequate control of the inflammatory response can be accompanied by a marked improvement in hemodynamics in certain types of PH.


2020 ◽  
Vol 22 (Supplement_F) ◽  
pp. F30-F37
Author(s):  
Stepan Havranek ◽  
Zdenka Fingrova ◽  
David Ambroz ◽  
Pavel Jansa ◽  
Jan Kuchar ◽  
...  

Abstract Atrial fibrillation (AF) and atrial tachycardia (AT) are frequently observed in patients with chronic thromboembolic pulmonary hypertension (CTEPH) who were treated with pulmonary endarterectomy (PEA). Their prevalence and impact on prognosis of patients are not known. We analysed the prevalence of AF/AT and the clinical outcome in 197 patients with CTEPH treated with PEA (median age 62; interquartile range 53–68 years; 62% males). The prevalence of AF/AT was 29% (57 patients). Compared to patients without arrhythmia, the subjects with AF/AT were older [60 (50–67) vs. 62 (57–70) years], manifested an increased size of the left atrium [39 (35–44) vs. 45 (40–50) mm], had a reduced 6-min walking distance [411 (321–506) vs. 340 (254–460) m], and higher pulmonary artery systolic pressure after PEA [38 (30–47) vs. 45 (38–71) mmHg], all results with P-value &lt;0.05. During the follow-up with a median 4.2 (1.6–6.3) years, 45 (23%) patients died. In a multivariate Cox regression model only the male gender [hazard ratio (HR) 2.27, 95% confidence interval (CI) 1.15–4.50], a reduced 6-min walking distance (HR 3.67, 95% CI 1.74–7.73), and an increased New York Heart Association class (HR 8.56, 95% CI 4.17–17.60) were associated with mortality (P &lt; 0.05). The prevalence of AF/AT in patients with CTEPH treated with PEA is high. Arrhythmias are associated with reduced functional capacity but not with mortality.


2017 ◽  
Vol 2017 ◽  
pp. 1-8
Author(s):  
Bingjie Wu ◽  
Jingjing Jiang ◽  
Minghui Gui ◽  
Lin Liu ◽  
Qiqige Aleteng ◽  
...  

The aim of this study was to evaluate the association between thyroid hormone levels, pulmonary hypertension (PH), and pulmonary artery systolic pressure (PASP) in euthyroid patients with coronary artery disease (CAD). A cross-sectional study was conducted in individuals who underwent coronary angiography and were diagnosed as CAD from March 2013 to November 2013. 811 subjects (185 women and 626 men) were included in this study. PASP was measured by transthoracic Doppler echocardiography. 86 patients were diagnosed as PH and had significantly higher free thyroxine (FT4) levels than those without PH. Multiple logistic regression analysis demonstrated an independent association of FT4 levels with PH after adjustment of gender, age, body mass index, systolic blood pressure, left ventricular ejection fraction, hypertension, and medication use of calcium channel blockers, ACE inhibitors, angiotensin II receptor antagonists, and nitrates. Serum-free triiodothyronine (FT3) and thyroid-stimulating hormone (TSH) were not associated with PH. Furthermore, multivariate linear regression analysis showed that FT4 levels emerged as an independent predictor for PASP, while FT3 and TSH levels were not associated with PASP. Our study demonstrated that, in euthyroid patients with CAD, FT4 was an independent risk factor for PH, and FT4 levels were independently associated with PASP.


Circulation ◽  
2015 ◽  
Vol 132 (suppl_3) ◽  
Author(s):  
Maria Drakopoulou ◽  
Konstantinos Stathogiannis ◽  
Konstantinos Toutouzas ◽  
George Latsios ◽  
Andreas Synetos ◽  
...  

Objective: Severe aortic stenosis leads to increased pulmonary arterial systolic pressure. A controversy still remains regarding the impact of persistent pulmonary hypertension (PHT) on prognosis of patients undergoing transcatheter aortic valve implantation (TAVI). We sought to investigate the impact of persistent PHT on 2-year all-cause mortality of patients with severe aortic stenosis following TAVI. Methods: Patients with severe and symptomatic aortic stenosis (effective orifice area [EOA]≤1 cm 2 ) who were scheduled for TAVI with a self-expanding valve at our institution were prospectively enrolled. Prospectively collected echocardiographic data before and after TAVI were retrospectively analyzed in all patients. Pulmonary artery systolic pressure was estimated as the sum of the right ventricular to the right atrial gradient during systole and the right atrial pressure. PHT following TAVI was classified as absent if <35 mmHg and persistent if ≥35 mmHg. Primary clinical end-point was 2-year all-cause mortality defined according to the criteria proposed by the Valve Academic Research Consortium-2. Results: Hundred and forty patients (mean age: 82±9 years) were included in the study. The primary clinical end point occurred in 17 patients (12%) during a median follow-up period of 2 years. Mean pulmonary artery systolic pressure was reduced in all patients following TAVI (45±9 versus 41±6 mmHg, p<0.01). Mortality rate was higher in patients with persistent PHT compared to patients with normal pulmonary artery systolic pressure following TAVI (26% versus 14 %, p<0.01). Patients that reached the primary clinical end point had a higher post procedural mean systolic pulmonary pressure (43±9 versus 39±6 mmHg, p=0.02). In multivariate regression analysis, persistence of PHT (OR: 2.51, 95% CI: 1.109-7.224, p=0.01) was an independent predictor of long-term mortality. Conclusions: The persistence of pulmonary hypertension after TAVI is associated with long term mortality. Identifying the population that will clearly benefit from TAVI is still need to be validated by larger trials.


1988 ◽  
Vol 10 (2) ◽  
pp. 59-63
Author(s):  
H. James Holroyd

As many as half of routine ill-patient visits in pediatrics practice involve respiratory symptoms. It is not surprising that we see so much coughing and wheezing when we consider that children are still developing immunologically and are, therefore, more susceptible to infection. In early life, most of these infectious diseases occur in the respiratory and gastrointestinal tracts. In addition, the gradual exposure of the constitutionally allergic child to environmental allergens pre-disposes to the development of respiratory symptoms. Childhood is also a time for congenital defects of the cardiopulmonary system to become apparent. A sometimes overlooked cause of respiratory symptoms in infants and young children—and one that can become rapidly and severely complicated if misdiagnosed—is the aspiration of foreign bodies. Aspiration or ingestion of foreign bodies remains a significant cause of morbidity and mortality in the pediatric population. Children between 8 months and 4 years of age are at highest risk. Not all cases of aspiration are of the acute, obstructive variety, and pediatricians should consider a foreign body as a possible cause of coughing and wheezing even when no definite history of aspiration is obtained. Children of course are curious and their natural tendency is to reach out and explore new objects. Bringing objects to their mouths to taste and to test textures is common.


Blood ◽  
2004 ◽  
Vol 104 (11) ◽  
pp. 1634-1634
Author(s):  
Victor R. Gordeuk ◽  
Adelina I. Sergueeva ◽  
Galina Y. Miasnikova ◽  
Lydia A. Polyakova ◽  
Daniel J. Okhotin ◽  
...  

Abstract Chuvash polycythemia is characterized by a homozygous 598C>T mutation in VHL and up regulation of HIF-1α during normoxia. Disorders of chronic hypoxia may be complicated by the development of pulmonary hypertension. Because of the up regulation of the hypoxic response in Chuvash polycythemia, we postulated that there may be a tendency to increased pulmonary artery pressures in this condition as well. To test this hypothesis, we analyzed results for Doppler echocardiography in 15 patients with Chuvash polycythemia and 15 Chuvash individuals without polycythemia. The tricuspid regurgitation velocity (TRV) allows estimation of pulmonary artery systolic pressure. A TRV of 2.5 m/sec or higher corresponds to a pulmonary artery systolic pressure of at least 35 mm Hg (normal up to 32 mm Hg), while a TRV of 3.0 m/sec or higher to a pressure of at least 46 mm Hg. The results are summarized in the Table. Pulmonary artery pressures as estimated by tricuspid regurgitation velocity (TRV) in Chuvash subjects with and without polycythemia Chuvash polycythemia (n = 15) Controls (n = 15) P Age in years; mean (SD) 35 (17) 35 (17) 1.0 Female sex in no. (%) 8 (53%) 8 (53%) 1.0 Hemoglobin in g/dL; mean (SD) 16.7 (2.3) 13.3 (1.2) <0.001 TRV in m/sec; mean (SD) 2.2 (0.6) 1.4 (0.6) 0.001 TRV > 2.4 m/sec in no. (%) 4 (27%) 0 (0%) 0.1 Most of the patients with Chuvash polycythemia were receiving phlebotomy therapy and therefore many had hemoglobin concentrations in the upper normal range. Four of the patients with Chuvash polycythemia and none of the others had TRV ≥ 2.5 m/sec (range of 2.5 to 3.0), and mean TRVs were significantly higher in the patients with Chuvash polycythemia. Interestingly, the mean ± SD TRV in these 15 patients with Chuvash polycythemia was identical to the mean ± SD TRV that was recently reported in 195 American patients with sickle cell disease (Gladwin et al, NEJM2004;350:886), another hematological condition with a tendency to pulmonary hypertension. While the pulmonary arterial pressures detected so far in Chuvash polycythemia patients are lower than those in patients with primary pulmonary hypertension, our results suggest that pulmonary hypertension may be an unrecognized complication of Chuvash polycythemia.


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