Prevention and early diagnosis of pulmonary hypertension

Abstract Background Pulmonary tumour thrombotic microangiopathy (PTTM) is a fatal disease in which tumour cells embolize to the pulmonary vasculature leading to pulmonary hypertension and right heart failure. Early diagnosis is essential for timely treatment which can reduce intimal pulmonary vascular proliferation and prolong survival, improve the symptoms. Due to rare occurrences and no clear diagnostic guidelines the disorder usually is found post-mortem. We present a review of this rare disease and a case of post-mortem diagnosed pulmonary tumour thrombotic microangiopathy in a young female. Case presentation 51 years old woman presented with progressively worsening dyspnea, right ventricular failure signs and symptoms. Computerized tomography denied pulmonary embolism. 2D transthoracic echocardiography demonstrated right ventricle dilatation and dysfunction, severely increased systolic pulmonary pressure. Right heart catheterization revealed pre-capillary pulmonary hypertension with mean pulmonary artery pressure of 78 mmHg, pulmonary wedge pressure of 15 mmHg, reduced cardiac output to 1.78 L/min with a calculated pulmonary vascular resistance of 35 Wood units, and extremely low oxygen saturation (26%) in pulmonary artery. Because of worsening ascites, pelvic magnetic resonance imaging was performed, tumours in both ovaries were diagnosed. Due to the high operative risk, detailed tumour diagnosis surgically was not established. The patient developed progressive cardiorespiratory failure, unresponsive to optimal heart failure drug treatment. A postmortem morphology analyses revealed tumorous masses in pre-capillary lung vessels, right ventricle hypertrophy, ovary adenocarcinoma. Conclusions An early diagnosis of PTTM is essential. Most cases are lethal due to respiratory failure progressing rapidly. Patients with a history of malignancy, symptoms and signs implying of PH should be considered of having PTTM. If detected early enough, combination of chemotherapy with specific PH therapy is believed to be beneficial in reducing intimal proliferation and prolonging survival, along with improving the symptoms.

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Pulmonary Veno-occlusive Disease

Advances in Pulmonary Hypertension ◽

10.21693/1933-088x-14.3.155 ◽

2015 ◽

Vol 14 (3) ◽

pp. 155-160

Author(s):

David Montani ◽

Barbara Girerd ◽

Marc Humbert

Keyword(s):

Risk Factors ◽

Pulmonary Hypertension ◽

Pulmonary Arterial Hypertension ◽

Early Diagnosis ◽

Arterial Hypertension ◽

Clinical Presentation ◽

Occlusive Disease ◽

Rare Form ◽

Pulmonary Arterial ◽

Specific Management

As a rare form of pulmonary hypertension, the pathophysiology and clinical presentation of pulmonary veno-occlusive disease (PVOD) has long remained poorly understood. In this review, we will discuss the distinctions between presentation of PVOD and pulmonary arterial hypertension, and address the importance of early diagnosis in proposing specific management. We will explore the risk factors and conditions associated with PVOD, and describe the challenges surrounding its diagnosis and management.

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Pulmonary Hypertension due to Lung Disease – Increasing need for the Early Diagnosis and Future Treatment Strategies

Journal of Cardiac Failure ◽

10.1016/j.cardfail.2013.08.113 ◽

2013 ◽

Vol 19 (10) ◽

pp. S124

Author(s):

Ichizo Tsujino

Keyword(s):

Pulmonary Hypertension ◽

Early Diagnosis ◽

Lung Disease ◽

Treatment Strategies ◽

Future Treatment

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Can computer assisted analysis of a standard 12‑lead ECG be used for early diagnosis and monitoring of disease progression in pulmonary hypertension?

International Journal of Cardiology ◽

10.1016/j.ijcard.2018.08.057 ◽

2018 ◽

Vol 273 ◽

pp. 221-222

Author(s):

Jens Erik Nielsen-Kudsk ◽

Stine Andersen ◽

Asger Andersen

Keyword(s):

Pulmonary Hypertension ◽

Early Diagnosis ◽

Disease Progression ◽

Computer Assisted ◽

Assisted Analysis

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Toward Early Diagnosis of Pulmonary Hypertension

Journal of the American College of Cardiology ◽

10.1016/j.jacc.2019.03.473 ◽

2019 ◽

Vol 73 (21) ◽

pp. 2673-2675 ◽

Cited By ~ 1

Author(s):

Bradley A. Maron ◽

Bradley M. Wertheim

Keyword(s):

Pulmonary Hypertension ◽

Early Diagnosis

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Alpha-2-Antiplasmin : A Fibrinolytic Peptide, could be a Potential Biomarker for Diagnosis of High Altitude Induced Pulmonary Hypertension

Defence Science Journal ◽

10.14429/dsj.67.11694 ◽

2017 ◽

Vol 67 (6) ◽

pp. 631

Author(s):

Prasanna K. Reddy ◽

Shajer M. Malik

Keyword(s):

Pulmonary Hypertension ◽

Early Diagnosis ◽

High Altitude ◽

Vascular Remodeling ◽

Diagnostic Marker ◽

Large Sample Size ◽

Moderate Altitude ◽

Therapeutic Decisions ◽

Potential Biomarker ◽

Pulmonary Arterial

<p class="p1">Prolonged exposure to high altitude is known to cause pulmonary vascular remodeling and pulmonary hypertension. Despite the advancements in diagnostics of pulmonary hypertension (PH), a potential biomarker for early diagnosis of PH progression is still lacking. High altitude induced pulmonary hypertension (HAPH) is great concern in mountain regions across the world. Early diagnosis and monitoring the disease progression are critical for therapeutic decisions. A fibrinolytic peptide, alpha-2-antiplasmin (α2AP) is known to cause tissue and vascular remodeling. Recently, elevated levels of α2AP were reported in different forms of PH; however its role in high altitude induced pulmonary hypertension is not yet known. To assess whether α-2AP can be used as diagnostic marker for HAPH, a cross sectional study was carried out on 100 healthy male Indian army soldiers who were posted to high altitude (14,800 ft to 18,700 ft) for a prolonged period of 6 to 8 months. We used 2D Echocardiography to screen the pulmonary arterial systolic pressure (PASP) and semi-quantitatively ELISA method for estimation of α-2AP in plasma. We found 34 out of 100 subjects have elevated PASP upon immediate screening after de-induction to moderate altitude of 10,000 ft. Age matched 17 subjects with elevated PASP were assessed for plasma levels of α-2AP in comparison to subjects with normal PASP, but posted to same altitude for same duration of time. Here for the first time we demonstrated an increase in plasma level of α-2AP was positively correlated with hypoxia induced elevated pulmonary arterial pressure. However, additional comprehensive investigations are warranted with large sample size to confirm our findings and explore the use of α2AP as potential diagnostic marker for monitoring progression of HAPH.<span class="Apple-converted-space"> </span></p>

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Early Diagnosis of Connective Tissue Disease-Related Pulmonary Hypertension

Japanese Journal of Clinical Immunology ◽

10.2177/jsci.32.457 ◽

2009 ◽

Vol 32 (6) ◽

pp. 457-465

Author(s):

Masanori FUNAUCHI ◽

Kazuya KISHIMOTO ◽

Koji KINOSHITA

Keyword(s):

Pulmonary Hypertension ◽

Connective Tissue ◽

Early Diagnosis ◽

Connective Tissue Disease

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Newborn with bilateral congenital cataracts: Never forget congenital rubella syndrome

Paediatrics & Child Health ◽

10.1093/pch/pxz005 ◽

2019 ◽

Vol 25 (2) ◽

pp. 72-76

Author(s):

Inara Chacon Fonseca ◽

Jacqueline Wong ◽

Kamiar Mireskandari ◽

David Chitayat

Keyword(s):

Pulmonary Hypertension ◽

Family History ◽

Early Diagnosis ◽

Signs And Symptoms ◽

Congenital Rubella Syndrome ◽

Persistent Pulmonary Hypertension ◽

Diagnostic Approach ◽

Congenital Cataracts ◽

Rare Finding ◽

Congenital Rubella

Abstract A term, growth-restricted newborn presented with a sepsis-like picture, persistent pulmonary hypertension and bilateral cataracts. Initial review of prenatal and family history, as well as microbiological investigations were noncontributory. Following lenticular extraction, viral examination of the lenses confirmed the presence of rubella. Congenital rubella syndrome (CRS) presents with a constellation of signs and symptoms that overlap with many other conditions. The presence of bilateral cataracts in a newborn is a rare finding and this case is used to review the broad etiology of congenital cataracts. We propose a structured diagnostic approach for clinicians, remembering that CRS is a rare but possible etiology. The early diagnosis of CRS in this case, allowed us to initiate appropriate management and preventive measurements.

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