Helping Patients Understand the Complex Pulmonary ...

1 PH Professional Network Helping Patients Understand the Complex Pulmonary Hypertension Workup Section Editor Martha Kingman, NP the evaluation of unexplained shortness of breath. The results may reveal obstructive and/or restrictive disease, which will allow Fran Rogers, MSN, CRNP they have taken diet pills or used meth- placement of the patient in the proper PH. Nurse Practitioner/Clinical Coordinator amphetamine and should be counseled re- classification and also guide Pulmonary Hypertension garding the importance of divulging this Program relevant information. Overnight Oximetry: This relatively sim- Hospital of the University ple test can be done in the patient's home.

2 A. of Pennsylvania The physical examination is one of the Philadelphia, PA most important aspects of the initial PH local oxygen supply company can provide evaluation. The patient can expect a fo- the necessary (and minimal) equipment. Pa- When Patients are being evaluated for cused cardiac examination that will pro- tients are asked to wear a pulse oximeter Pulmonary hypertension (PH), they are vide information about the functioning of while sleeping for one night. Results can often asked to undergo numerous diag- the right side of the heart. The patient identify the degree and frequency oxygen nostic tests in order to reach a definitive should know that this will be a part of levels drop during sleep.

3 Based on results, diagnosis. An important role of the PH every subsequent examination. the patient may need to wear nocturnal ox- nurse coordinator is educating the Patients ygen or have a formal sleep study to eval- regarding the purpose and importance of Electrocardiogram (ECG): Patients may uate for obstructive sleep apnea. the various procedures. ask why an ECG is being performed. This is Laboratory Tests: The patient will require The classification of PH has gone a quick, noninvasive way to assess electro- very specific laboratory work when seen at through a series of changes since the first mechanical changes occurring in the right the dedicated PH center.

4 Included in a wide classification in The current guide- heart. Their first ECG will serve as a base- variety of labs will be brain natriuretic pep- lines recommend certain diagnostic testing line for comparison with future ECGs. tide (BNP) or NT-pro-BNP. Elevations of be performed on Patients suspected of hav- either can alert clinicians to the presence of ing PH in order to provide diagnostic clar- Transthoracic Echocardiogram (TTE): right ventricular strain and possible right ity. The American College of Cardiology The patient needs to know that the diagnosis heart The patient should know that Foundation (ACCF)/American Heart Asso- of PH cannot be made on the basis of an following trends could provide valuable in- ciation (AHA) 2009 Expert Consensus echocardiogram This noninvasive formation regarding effectiveness of PH- Document attests to the complexity of diag- test can, however, provide important infor- specific therapies.

5 Additionally, laboratory nosing and caring for Patients with PH (Fig- mation about the size and function of the tests may be ordered to evaluate for the ure).2 One important recommendation is right ventricle, as well as evaluate for con- presence of connective tissue disease, as that Pulmonary arterial hypertension (PAH) genital heart disease or left sided heart fail- well as HIV and hepatitis B and C. Patients be evaluated and treated in conjunc- ure. Using a heart model can be an excellent tion with a PH center of The visual way of illustrating changes that can 6-Minute Walk Test (6 MWT): The pa- following are some of the recommendations occur to the right side of the heart.

6 Tient should know this is a simple test for the PH workup. The TTE can also allow for specific mea- requiring a walk on a flat, hard surface for surements, such as tricuspid annular plane 6 Speed, distance, and oxygen- History and Physical Examination: Pa- systolic excursion (TAPSE), which can help ation levels will be measured. Results pro- tients need to Understand the value of an predict survival in these Patients vide a good sense of functionality and accurate history and thorough physical should know that a TTE will be repeated at oxygen requirements with exertion. Sub- examination. Inviting a family member certain intervals based on clinical presenta- sequent walk test results can help in the who is familiar with the patient's course tion, as well as the individual PH center's assessment of disease progression as well of illness can be extremely helpful, par- routine for repeating TTEs.

7 As response to therapy. ticularly if the patient has difficulty pro- viding an accurate history. Pulmonary Function Tests (PFTs): The Right Heart Catheterization (RHC): Fatigue and dyspnea are the symptoms results of PFTs will provide important in- The patient should know that a RHC will be Patients most often Thoughtful formation on ventilatory function as well as required to establish the diagnosis of questioning of the patient and family may gas exchange. They can also be helpful in They should be educated on how this inva- reveal the presence of chest pain, short- ness of breath, palpitations, presyncope, Correspondence: or syncope.

8 Patients are often asked if Advances in Pulmonary Hypertension 65. Pivotal Tests Contingent Tests Contribute to Assessment of: History Exam Index of Suspicion of CXR PH. ECG. RVE, RAE, RVSP, RV. TEE Function Echocardiogram Exercise echo Left Heart Disease VHD, CHB. Pulmonary Angiography Chest CT Anglogram Chronic PE. VQ Scan Coagulopathy Profile Ventilatory Function PFTs ABGs Gas Exchange Overnight Sleep Disorder Polysomnography Oximetry HIV HIV Infection ANA Other CTD Serologies Scleroderma, SLE, RA. LFTs Portopulmonary Htn Functional Test Establish Baseline (6 MWT,CPET) Prognosis Vasodilator Test Confirmation of PH.

9 RH Cath Exercise RH Cath Hemodynamic Profile Volume Loading Vasodilator Response Left Heart Cath Figure: Diagnostic approach to General guidelines for the evaluation of Pulmonary hypertension. Since the suspicion of PH may arise in various ways, the sequence of tests may vary. However, the diagnosis of PAH requires that certain data support a specific diagnosis. In addition, the diagnosis of idiopathic Pulmonary arterial hypertension is one of excluding all other reasonable possibilities. Pivotal tests are those that are essential to establishing a diagnosis of any type of PAH either by identification of criteria of associated disease or exclusion of diagnoses other than IPAH.

10 All pivotal tests are required for a definitive diagnosis and baseline characterization. An abnormality of one assessment (such as obstructive Pulmonary disease on PFTs), does not preclude that another abnormality (chronic thromboembolic disease on VQ scan and Pulmonary angiogram) is contributing or predominant. Contingent tests are recommended to elucidate or confirm results of the pivotal tests, and need only be performed in the appropriate clinical context. The combination of pivotal and appropriate contingent tests contribute to assessment of the differential diagnoses in the right-hand column.