Pulmonary hypertension symptoms

During COPD, the following symptoms occur, usually in the order mucus hypersecretion, ciliary dysfunction, airflow limitation, pulmonary hyperinflation, gas exchange abnormalities, pulmonary hypertension and cor pulmonale. Acute exacerbations appear to be mainly triggered by bacteria, viruses or environmental pollutants. They lead to a worsening of lung functions, wasting and increased mortality their psychosocial impacts include depression and anxiety that may be associated with the will to die. 

MDMA overdose as well as the concomitant consumption of selective serotonin reuptake inhibitors (SSRI) with other dmgs that exert serotoninergic effects (such as inhibitors of monoamine oxidase) can rapidly lead to the serotonin syndrome. Its symptoms, which are reversible upon cessation, of the drug include confusion, muscle rigidity in the lower limbs, and hyperthermia suggesting an acute reaction to serotonin overflow in the CNS. Blocking the function of SERT outside the brain causes side effects (e.g., nausea), which may be due to elevated 5HT however , impairment of transporter function is not equivalent to direct activation of 5HT recqrtors in causing adverse effects such as fibrosis and pulmonary hypertension. 

Commonly found in asbestosis are bilateral end-inspiratory pulmonary rales at the lung bases, dyspnea, finger clubbing, and cyanosis, but any or all of these symptom can be absent in any one case. Pulmonary hypertension is frequently associated with advanced asbestosis, and the resultant cor pulmonale (right-sided heart failure) may be the cause of death (Lemen et al., 1980, p. 2). 

Mecfianism of Action A prostaglandin that dilates systemic and pulmonary arterial vascular beds, alters pulmonary vascular resistance, and suppresses vascular smooth muscle proliferation. Therapeutic Effect Improves symptoms and exercise tolerance in patients with pulmonary hypertension delays deterioration of condition. Pharmacokinetics Protein binding 60%. Metabolized in liver. Primarily excreted in urine minimal elimination in feces. Half-life 20-30 min. 

Pulmonary hypertension in patients with NYHAClass III or IV symptoms Oral Inhalation Initially, 2.5 mcg/dose if tolerated, increased to 5 mcg/dose. Administer 6-9 times a day at intervals of 2 hr or longer while patient is awake. Maintenance 5 mcg/dose. Maximum daily dose 45 meg. 

Abrupt withdrawal or sudden large reductions in dosage may result in worsening of pulmonary arterial hypertension symptoms. 

There has been a sequential comparison of inhaled nitric oxide 40 ppm with aerosolized iloprost 14— 17 micrograms in 35 adults with primary pulmonary hypertension (125). Five of the patients had minor headache and facial flushing during inhalation of iloprost, but these symptoms were short-lived and abated a few minutes after the inhalation ended. One patient had mild jaw pain after aerosolized iloprost, but again this was shortlived. There was an unexpected increase in pulmonary artery pressure in 10 patients and vascular resistance in six patients who received nitric oxide. The authors were uncertain of the cause of this increase, as nitric oxide generally behaves as a vasodilator, but they noted that... 

Prostacyclin lowers peripheral, pulmonary, and coronary resistance. It has been used to treat both primary pulmonary hypertension and secondary pulmonary hypertension, which sometimes occurs after mitral valve surgery. A commercial preparation of prostacyclin (epoprostenol) is approved for treatment of primary pulmonary hypertension, in which it appears to improve symptoms and prolong survival. However, because of its extremely short plasma half-life, the drug must be administered as a continuous intravenous infusion through a central line. Several prostacyclin analogs with longer half-lives have been developed and treprostinil was recently approved for use in pulmonary hypertension (Horn, 2002). This drug is administered by continuous subcutaneous infusion. 

Progressive pulmonary hypertension occurred in two patients who took fenfluramine for only 8 months (SEDA-6, 9). The symptoms abated on withdrawal but returned in one patient when rechallenged. 

The cyclo-oxygenase metabolite prostacyclin is a potent, short-lived vasodilator and antithrombotic agent. Intravenous administration of the commercially available form of prostacyclin, epoprostenol, relieves the symptoms of primary pulmonary hypertension by dilating the pulmonary vasculature [99]. A stable prostacyclin analogue, iloprost, appears to be similarly effective when administered as an aerosol and obviates the logistical problems associated with maintained intravenous administration [100]. 

Patients with pulmonary hypertension often complain of exertional dyspnea, chest pain, and syncope. Due to the nonspecific nature of these symptoms and lack of a noninvasive diagnostic test for detecting pulmonary hypertension, there are often delays in the... 

In a 12-year observational study, 62 patients with fenfluramine-associated pulmonary hypertension were compared with 125 sex-matched patients with pulmonary hypertension unrelated to the use of fenfluramine derivatives. In most of the cases (81%), fenfluramine derivatives were used for at least 3 months. The time frame between the initiation of the therapy and the onset of dyspnea ranged from 27 days to 23 years. Both the fenfluramine-associated pulmonary hypertension group and the control group had similar levels of New York Heart Association functional class and symptoms, as well as an overall survival rate of 50% in 3 years. ... 

Symptoms of pulmonary hypertension include dyspnea on exertion, chest pain, palpitations, syncope, ascites and lower extremity edema. Anginal symptoms may occur and are thought to be due to increased RV oxygen demand, decreased right coronary artery perfusion due to decreased pressure difference between aorta and RV end diastolic pressure, and rare instances of compression of the left main coronary artery by the dilated pulmonary trunk. Right ventricular hypertrophy (RVH) correlates with EKG findings including RV... 

For the growing number of patients with combined pulmonary hypertension and abnormal left ventricular hemodynamics, a careful hemodynamic study can help to delineate the subtleties of both diseases and response to therapies. Exercise catheterization is recommended in those patients with normal hemodynamics at rest, but with a pretest likelihood of PAH and/or other data suggesting exercise-induced symptoms, for instance, exercise echo or cardiopulmonary stress test. Unfortunately, to date there is no consensus as to the best exercise protocols for an appropriate hemodynamic assessment. Among those used include upright bicycle with neck pulmonary arterial (PA) lines at 75% predicted maximum exercise, supine bicycle, supine arm exercise, and supine volume loading. In all cases, it is essential to carefully measure PCWP, cardiac outputs, and PA pressures at consistent parts of the respiratory cycle, and not merely PA pressures. 

Usually the cause of pulmonary hypertension can be assigned to related parenchymal lung disease, heart disease, thromboembolism, or pulmonary, vascular disease. Pulmonary hypertension is termed idiopathic (or primary), however, when it occurs in patients in the absence of associated cardiopulmonary disease and when no other apparent cause for the disease is discernible. Primary pulmonary hypertension is a very rare disease that occurs predominantly in young female patients between the ages of 20 to 40 years (Wood, 1956). It is usually progressive and fatal, with the average survival time from the onset of symptoms being 2 to 3 years (Bourdillon and Oakley, 1976). 

The majority of patients with pulmonary hypertension are largely asymptomatic until marked vascular alterations have developed. When blood flow through the pulmonary artery is obstructed over a long period of time, however, the clinical picture is predictable and markedly uniform. In general, the patients exhibit normal pulmonary function measurements, a low carbon monoxide diffusion capacity (DlCO), and marked hyperventilation that leads to hypocapnia and decreased serum bicarbonate concentrations. Additional symptoms include weakness, fatigue, exertional dyspnea, and chest pains upon exertion due to low cardiac output and hypoxemia. Occasionally, hoarseness, hemoptysis, and cyanosis occur. 

Pulmonary hypertension is a rare idiopathic disease that mainly affects young adults. It leads to right-sided heart failure and frequently is fatal. Long-term therapy with PGI2 (epoprostenol, Flolan) has either delayed or precluded the need for lung or heart-lung transplantation in a number of patients. In addition, many affected individuals have had a marked improvement in symptoms after receiving treatment... 

---