Do the Different Types of Pulmonary Hypertension Cause the Same Effect on Right Heart and Serum Asymmetrical Dimethylarginine Levels in Female Patients?

ABSTRACT Objective: Right-heart function is a major determinant of clinical outcome in patients with elevated pulmonary artery pressure due to pulmonary venous hypertension (PVH) and pulmonary arterial hypertension (PAH). Asymmetric dimethylarginine (ADMA) is an endogenous inhibitor of nitric oxide synthase. This study aimed to evaluate if different types of pulmonary hypertension (PH) would cause the same effect on right-heart functions and serum ADMA levels in female patients. Methods: This study included patients with PAH as group I, patients with PVH due to mitral stenosis (mitral valve area ≤ 1.5 cm2, without any additional valve or left-heart disease and systolic pulmonary artery pressure ≥ 50 mmHg in transthoracic echocardiography) as group II, and healthy control subjects as group III. Transthorasic echocardiographic evaluations for right-heart functions were performed according to the guidelines of the American Society of Echocardiography. Venous blood samples were collected, and the serum ADMA concentrations were obtained with the ELISA kit (DRG® International Inc., Springfield, NJ, USA). Results: Patients in groups I and II had higher ADMA levels than healthy control subjects. Right-atrium area and dimensions, right-ventricular (RV) volumes, grade of tricuspid regurgitation, systolic pulmonary arterial pressure, RV wall thickness, and RV outflow tract diameters were significantly higher in group I patients than in group II patients. Right-ventricular myocardial performance index was lower, and RV fractional area change and tricuspid valve systolic tissue Doppler velocity were higher in group II patients than in group I patients. Conclusion: This study demonstrated that both PAH and PVH caused increase in right-heart dimensions and impairment in right-heart functions.

Combination Therapy in Pulmonary Arterial Hypertension: Single Centre Long-term Experience.

BACKGROUND: Pulmonary arterial hypertension (PAH) is a progressive disease of the pulmonary vascular bed and causes right heart failure and death. Combination therapy which targets three different pathways is necessary due to the progressive nature of the disease. In patients with PAH, there are two approaches in combination therapy: "first-line up-front" and "sequential add-on" treatment. In "firstline up-front" treatment, patients receive double or triple drug therapy from the start. In the "sequential add-on" approach, a single drug is initially started and then according to the patient's requirements, a second or third drug is added. There is insufficient evidence about the efficiency and safety of treatment approaches. In this study, we aimed to evaluate the treatment approach in patients with PAH at a tertiary centre. METHODS: Pulmonary arterial hypertension was diagnosed according to clinical, echocardiographic and right heart catheterization findings. The patients received bosentan, sildenafil and iloprost treatment in accordance with guidelines recommendations. Clinical worsening in patients was defined as death, requirement of hospitalization for PAH, a 15% decline in the six-minute walk test (6MWT) distance, deterioration in functional capacity, and symptoms and findings of right heart failure. RESULTS: At the end of the follow-up period, clinical and echocardiographic findings, brain natriuretic peptide (BNP) levels and oxygen saturation were similar between patients who completed the study with monotherapy and with combination therapy. The follow-up period was significantly longer in patients who required combination treatment. Two patients (6.9%) died and four patients (13.8%) were hospitalized due to recurrent symptoms and findings of right heart failure. At the end of follow-up, 10 patients (34.5%) completed the study with a single drug, 15 patients (51.7%) with two drugs and four patients (13.8%) with three drugs. CONCLUSION: In this study, combination therapy was given to patients as "sequential add-on therapy". At the end of the follow-up period, monotherapy was sufficient in 34.5% of patients of the study group and in eight patients, sildenafil or prostaglandin analogues were added; a total of 15 patients (48.4%) completed the study under dual therapy. Four patients (12.9%) received combination therapy with three drugs.

Successful switch from inhaler iloprost to treatment in a patient who initially presented with pulmonary arterial hypertension and diagnosed as SLE.

BACKGROUND: SLE-induced Pulmonary Arterial Hypertension increases morbidity and mortality. Vasoconstruction, thrombosis, inflammation are the mostly proposed factors in PAH. Time until PAH develops is variable in patients with SLE. It has been shown that vasoactive agents improve symptoms and clinical findings in patients with SLE-induced-PAH. METHODOLOGY: The case note of the patient with with pulmonary arterial hypertension and SLE was retrieved and reviewed, and literature search was done. We reported a patient whose initially symptoms and clinical findings were consistent with idiopathic PAH and then had been diagnosed as SLE. RESULT: The patient tolerated the switch treatment from inhaler Iloprost from oral Bosentan well. Her symptoms, clinical findings and 6 minutes walking distance improved with treatment. CONCLUSION: Vasodilator treatment appears to be beneficial in patients with SLE-associated PAH and switch therapy also seems to be well tolerated. Additionally, in connective tissue diseases it must be kept in mind that PAH might be the presenting symptom.