The Relationship Between Retinal Microcirculation and Right Heart Catheterization and Echocardiography Findings in Patients with Idiopathic Pulmonary Arterial Hypertension

2021 ◽  
Author(s):  
Vedat Hekimsoy ◽  
Hilal Kılınç Hekimsoy ◽  
Mehmet Ali Şekeroğlu ◽  
Ergün Barış Kaya ◽  
Lale Tokgözoğlu
2018 ◽  
Vol 05 (02) ◽  
pp. 107-109
Author(s):  
Jamir Pitton Rissardo ◽  
Ana Letícia Fornari Caprara

AbstractPulmonary arterial hypertension (PAH) is a progressive pulmonary vasculopathy. A 29-year-old female patient presenting with dyspnea and syncope within 6 hours of onset was admitted to our hospital. The patient stated that she looked for a neurologist months ago because she experienced abrupt shaking limbs occurring during physical activity. She was diagnosed with focal seizure, and carbamazepine (CBZ) was started. On admission, she reported that the dyspnea had started in the last week and recurrent episodes of syncope in the last few hours. A right heart catheterization was diagnostic of PAH. She was started on spironolactone, furosemide, sildenafil, warfarin, and supplemental oxygen. On 10th admission day, the patient was seizure free and the dose of CBZ was tapered. In the follow-up, the patient remained seizure free. An investigation to search for a chronic lung disease or hypoxemia, systemic disorder, hematological disorder, and metabolic disorder was negative.


2020 ◽  
Vol 11 ◽  
Author(s):  
Jean Pierre Sibomana ◽  
Aloma Campeche ◽  
Roberto J. Carvalho-Filho ◽  
Ricardo Amorim Correa ◽  
Helena Duani ◽  
...  

Pulmonary arterial hypertension (PAH) is a disease of the lung blood vessels that results in right heart failure. PAH is thought to occur in about 5% to 10% of patients with hepatosplenic schistosomiasis, particularly due to S. mansoni. The lung blood vessel injury may result from a combination of embolization of eggs through portocaval shunts into the lungs causing localized Type 2 inflammatory response and vessel remodeling, triggering of autonomous pathology that becomes independent of the antigen, and high cardiac output as seen in portopulmonary hypertension. The condition is likely underdiagnosed as there is little systematic screening, and risk factors for developing PAH are not known. Screening is done by echocardiography, and formal diagnosis requires invasive right heart catheterization. Patients with Schistosoma-associated PAH show reduced functional capacity and can be treated with pulmonary vasodilators, which improves symptoms and may improve survival. There are animal models of this disease that might help in understanding disease pathogenesis and identify novel targets to screen and treatment. Pathogenic mechanisms include Type 2 immunity and activation and signaling in the TGF-β pathway. There are still major uncertainties regarding Schistosoma-associated PAH development, course and treatment.


2011 ◽  
Vol 39 (1) ◽  
pp. 100-105 ◽  
Author(s):  
HERMAN M.A. HOFSTEE ◽  
ALEXANDRE E. VOSKUYL ◽  
ANTON VONK NOORDEGRAAF ◽  
YVO M. SMULDERS ◽  
PIET E. POSTMUS ◽  
...  

Objective.Impaired microvascular function may contribute to organ complications in patients with systemic sclerosis (SSc). We investigated whether SSc patients with and without pulmonary arterial hypertension (PAH) show a graded impairment of microvascular function compared to healthy controls.Methods.Twenty-two patients with SSc and 22 controls were studied. All patients underwent right heart catheterization; 6 had no PAH (SSc-nonPAH) and 16 had PAH (SSc-PAH). Acetylcholine (ACh)-mediated endothelium-dependent vasodilatation and sodium nitroprusside (SNP)-mediated endothelium-independent vasodilatation were assessed by iontophoresis combined with laser Doppler flowmetry.Results.Compared to sex- and age-matched controls, ACh-mediated vasodilatation was reduced in SSc-PAH (340.4% vs 79.5%, respectively; p < 0.01), but not in SSc-nonPAH (340.4% vs 397.9%; p = 0.90). No significant differences were present between the groups in SNP-mediated vasodilatation.Conclusion.Systemic microvascular endothelium-dependent vasodilatation is markedly reduced in SSc complicated by PAH.


Author(s):  
Masashi Yokose ◽  
Takashi Tomoe ◽  
Takehiko Yamaguchi ◽  
Takanori Yasu

Abstract Background There is an increasing number of elderly patients with pulmonary arterial hypertension, and their characteristics differ from those of young or middle-aged patients with this condition. Case Summary A 73-year-old woman with a history of myocardial infarction and cardiovascular risk factors was admitted to the hospital with 2-week exertional dyspnoea. Her initial diagnosis was heart failure with preserved ejection fraction, but the symptoms persisted despite receiving treatment with diuretics. Additional tests showed a significant decrease in diffusing capacity of carbon monoxide and findings suggestive of severe pulmonary hypertension. Contrast-enhanced computed tomography of the chest, and pulmonary angiography, showed no narrowing or obstruction of the pulmonary arteries. Right heart catheterization revealed hemodynamic data implying pre-capillary pulmonary hypertension. Her condition gradually deteriorated to World Health Organization functional class IV, and sequential combination therapy with tadalafil, macitentan, and selexipag was initiated with a pulmonary arterial hypertension diagnosis; however, she died 1 month later. Pathological findings in autopsy were consistent with pulmonary arterial hypertension, and some parts of the lungs revealed the presence of obstructive and interstitial lung disease. Discussion The majority of elderly patients with pulmonary arterial hypertension might have multimorbidity. However, there is no specific treatment strategy. It is associated with diagnostic delay and worse prognosis; therefore, early suspicion and comprehensive tests, including right heart catheterization, are essential for better management.


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