3) Maacy Traning Module-Asthma Symtoms & Diagnosis.pptx
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Maacy Medical Training PAH Symptoms AND Diagnosis Diagnosis of PAH Diagnosis should be based on: I. Symptoms II. Past medical history III. Family history IV. Physical examination V. Investigations Diagnosis of PAH I. Symptoms of PAH What Are the Symptoms of Pulmonary Arterial Hypertensio...
Maacy Medical Training PAH Symptoms AND Diagnosis Diagnosis of PAH Diagnosis should be based on: I. Symptoms II. Past medical history III. Family history IV. Physical examination V. Investigations Diagnosis of PAH I. Symptoms of PAH What Are the Symptoms of Pulmonary Arterial Hypertension? • Increased shortness of breath • Fatigue • Edema, or swelling of the feet, legs and eventually the abdomen and neck • Dizziness and fainting spells • Chest pain • Heart palpitations (racing or pounding) • Lips and fingers turning blue • Cough • Hoarseness Symptoms are non-specific and are commonly attributed to other conditions Over time, symptoms become more severe and limit normal daily activities Lungs with and without PAH I. Symptoms and Signs of PAH What CLINICAL SIGNS • Jugular venous distension • Prominent P2 • Right ventricle heave • Tricuspid insufficiency murmur • Right side S3 heart sound • Hepatomegaly • Peripheral edema II. Past Medical History • The clinical manifestations of pulmonary arterial hypertension (PAH) are frequently masked by the underlying disease entities. Obtaining a careful history may help differentiate PAH from groups 2-5 pulmonary hypertension. Important clues to a specific cause include the following: 1. History of heart murmur 1. Heavy snoring 2. Deep venous thrombosis (DVT) or pulmonary 2. Daytime hypersomnolence embolism (PE) 3. Raynaud phenomenon 4. Arthritis or arthralgias 5. Rash 6. Heavy alcohol consumption 7. Hepatitis 3. Morning headaches 4. Morbid obesity 5. Family history of pulmonary hypertension 6. Drug use, in particularly diet drugs and illicit drugs 7. Medications III. Family History • Each normal person has a pair of BMPR2 genes in each cell in our bodies. One copy is inherited from our father and the other is inherited from our mother. The copy of the BMPR2 gene which we inherit from each parent occurs by random chance, like flipping a coin. A mutation in only one copy (from mother or father) of the pair of BMPR2 genes is enough to cause FPAH in a child. • By simple observation it can be seen that any person in the bloodline of a family with FPAH has an overall risk of about 1 in 10, or 10%, of developing FPAH during their lifetime. • When a parent has a BMPR2 gene mutation, each child has a 50% chance to inherit the abnormal gene, and a 50% chance to inherit the normal gene. If a child inherits the normal gene, then that child’s risk is similar to that of the general population, which is about one in a million for developing PAH. IV. Physical Examination • The intensity of the pulmonic component of the second heart sound (P2) may be increased and the P2 may demonstrate fixed or paradoxic splitting. A systolic ejection murmur may be heard over the left sternal border. The murmur may be augmented by inspiration. A right ventricular heave may be palpated. • A prominent A wave may be observed in the jugular venous pulse. A right-sided fourth heart sound (S4) with a left parasternal heave may be auscultated. • Right ventricular failure leads to systemic venous hypertension and cor pulmonale. The signs of right ventricular failure include a high-pitched systolic murmur of tricuspid regurgitation, hepatomegaly, a pulsatile liver, ascites, and peripheral edema. In this scenario, a right ventricular third heart (S3) sound is also heard. • Signs of underlying cardiac, pulmonary, hepatic, or collagen-vascular disease are often present. • Patients with pulmonary arterial hypertension (PAH) often develop cor pulmonale, which further worsens hypoxemia and perpetuates pulmonary hypertension. V. Investigations Blood Tests Blood tests check the oxygen levels in the blood, they observe liver and kidney function, and they identify whether the patient has collagen vascular disease, thyroid problems, signs of infection or HIV antibodies. Chest XRays Chest X-rays can reveal an enlarged right ventricle or pulmonary arteries. Chest X-rays can also show signs of emphysema or scarring (interstitial fibrosis) of the lungs. • Increase RA/RV/PA size • Pruning of peripheral vessels Electrocar diogram (ECG) An electrocardiogr am checks the electrical impulses of the heart. However, an ECG alone is not enough to indicate a PH diagnosis Echocardio gram This painless procedure is often used to make a preliminary diagnosis by estimating the pressures in the right heart and assessing how well the heart is functioning. • Signs of PH (increased systolic pulmonary arterial pressure (sPAP), enlarged RA/RV Congenital heart defects may be present Pulmonar y Function Tests These tests measure how much air your lungs can hold, how much air moves in and out of them and the lungs’ ability to exchange oxygen. These tests may be performed to potentially identify its cause. • Spirometry/ PFT impairment normal or mildly impaired • SixMinute Walk Test During this test, a patient will be asked to perform an exercise, most commonly a six-minute walk. The purpose is to identify the patient’s exercise tolerance level. Nuclear Scan This diagnostic tool tests for blood clots in the lungs by producing a picture of air and blood flow to the lungs. A small dose of radioactive material is breathed in and another small dose is injected via a blood vessel into the lungs. Right-Heart Catheteriza tion If the results of initial tests point to PH, your doctor will schedule a rightheart catheterization (commonly referred to as a “right heart cath”). Rightheart catheterization is one of the most accurate and useful tests to get a definitive diagnosis for pulmonary hypertension Six-Minute Walk Test Exercise capacity: The 6-minute walking test (6MWT) A recent investigation showed that the best absolute-threshold values for 1 year mortality and 1 year survival, respectively, were 165 m and 440 m, respectively. Right-Heart Catheterization • Right heart catheterization is the gold standard for diagnosing and classifying PH PAH Severity and Risk assessment World Health Organization classification of functional status of patients with pulmonary hypertension Worsening WHO-FC is one of the most alarming indicators of disease PAH Severity and Risk assessment After the WHO Functional class classification and track the treatment effectiveness start with those assessment PAH Severity and Risk assessment 2015 ESC/ERS three-strata, riskassessment tool PAH Severity and Risk assessment PAH Severity and Risk assessment Suggested assessment and timing for the follow-up of patients with pulmonary arterial hypertension Patient-reported outcome measures (PROM) • Pulmonary Hypertension Outcome Review Questionnaire [CAMPHOR] • The emPHasis-10 score • Living with Pulmonary Hypertension Questionnaire (LPHQ) • Pulmonary Arterial Hypertension-Symptoms and Impact Questionnaire [PAH-SYMPACT]) Thank You