pulmonary hypertension patient case report

How is pulmonary arterial hypertension diagnosed in right heart failure?

Right heart catheterization demonstrated a mean pulmonary artery pressure (MPAP) of 58 mmHg with a pulmonary capillary wedge pressure (PCWP) of 7 mmHg, thereby supporting a diagnosis of pulmonary arterial hypertension. Bosentan, diuretics, and warfarin were initiated and the patient was discharged home.

What is the pathophysiology of pulmonary hypertension?

The unique pathophysiology needs to be understood if the clinician is going to manage this patient safely until they reach their pulmonary hypertension specialist. Pulmonary hypertension is most often encountered in the ED in patients with left heart failure or severe COPD. It is defined as an elevated pulmonary artery pressure > 25 mmHg.

What is a 7 Crit case for pulmonary hypertension?

CritCases 7 Pulmonary Hypertension – A Fine Balance. Ground EMS finds the patient to have severe shortness of breath with peripheral cyanosis and an oxygen saturation of 75%, respiratory rate of 30 on her 8 L oxygen via nasal prongs. When switched to a non-rebreather, her saturation increases to 88%.

What happened to the 57 year old woman with pulmonary hypertension?

Submit your comments to the author (s). The patient is a 57-year-old woman with a history of pulmonary hypertension who presented to the outpatient pulmonary clinic for a second opinion concerning her worsening dyspnea on exertion. She had been hospitalized two years prior with shortness of breath.

What is PAH in medical terms?

Pulmonary arterial hypertension (PAH) is a rare and devastating disease characterized by progressive increases in pulmonary arterial pressure and pulmonary vascular resistance that eventually lead to right ventricular failure and death.[1] Many patients with PAH are of childbearing age; however, pregnancy in the setting of PAH is contraindicated because of the high maternal–fetal morbidity, even with novel medical therapies that have improved outcomes.[2] Efficacy data indicated that treprostinil, a prostacyclin analog, significantly improves the exercise capacity of patients with PAH and may provide survival benefits. [3,4]The primary mechanism of action of treprostinilis reduction of pulmonary artery pressure (PAP) through direct vasodilation of the pulmonary and systemic arterial vascular beds, thereby improving systemic oxygen transport and increasing cardiac output with minimal alterations of the heart rate. [5,6]

What is the best treatment for PAH?

Given the astoundingly high mortality rate of patients with PAH, pregnancy is not recommended in these patients. If patients do become pregnant and decline fetal termination, they are treated with PDE-5 inhibitors, prostanoids, diuretics, and supplemental oxygen as needed.[2] Other treatments such as endothelin and nitric oxide are contraindicated because they cause fetal malformations. Clinical practice guidelines recommend oral PDE inhibitor therapy for mild to moderate PAH. [7,8]The mainstay of treatment for severe PAH is prostanoids. Prostanoids include epoprostenol, treprostinil, and beraprost. However, epoprostenol was withdrawn from the Chinese market in late 2016 because of external factors, that is, its use was not approved by health insurance.

Who published the book The Authors?

Copyright © 2018 The Authors. Published by Wolters Kluwer Health, Inc. All rights reserved.

What is pulmonary hypertension?

It is defined as an elevated pulmonary artery pressure > 25 mmHg. The pulmonary circulation is normally a low pressure, low resistance circuit.

What are the implications if this pulmonary hypertension patient were to have a cardiac arrest?

Patients with pulmonary hypertension and right heart failure who suffer cardiac arrest have an extremely poor prognosis. CPR in the event of a cardiac arrest is essentially futile given the obstructive physiology of severe pulmonary hypertension. Of course, patient and family wishes need to be incorporated into decision making.

What initial advice would you give to the community physician while you are en route for this patient with pulmonary hypertension?

First – Call pulmonary service ASAP. People do entire fellowships in the management of pulmonary hypertension.

What factors must be considered when treating this patient with shock and respiratory failure given their history of pulmonary hypertension and right heart failure?

The management of patients with pulmonary hypertension is highly specialized. The unique pathophysiology needs to be understood if the clinician is going to manage this patient safely until they reach their pulmonary hypertension specialist.

What are possible causes of this pulmonary hypertension patient’s decompensation?

Any condition that will cause a decrease in systemic blood pressure, increase in pulmonary arterial pressure or a combination thereof can cause an acute decompensation in these patients and initiate the death spiral of death.

What is your ventilation strategy in the patient with pulmonary hypertension?

Unlike an ARDS protocol for ventilation, these patients cannot tolerate permissive hypercapnea and hypoxia due to pulmonary vasoconstriction.

What other treatments are potentially available at a tertiary center for these pulmonary hypertension patients?

Failing inotropic and vasopressor support, these patients may require inhaled pulmonary vasodilator therapies, likely unavailable in a community hospital. Inhaled nitric oxide or inhaled epoprostenol provide pulmonary vasodilation while limiting systemic hypotension. Additionally, they may help to correct V/Q mismatch by preferentially vasodilating pulmonary arteries perfusing well ventilated lung. ECMO would remove deoxygenated blood from the venous system and restore oxygenated blood to the arterial system, which would benefit both right and left ventricular dysfunction as well as improve oxygenation.

How much mortality is there after pulmonary endarterectomy?

Thirty-day mortality following pulmonary endarterectomy has been estimated to be between 5-10% and appears to be dependent on the level of expertise of the performing center. Most postoperative mortality is related to reperfusion pulmonary edema and residual pulmonary hypertension. Pulmonary artery steal syndrome is a phenomenon caused by redistribution of blood flow to newly patent pulmonary vasculature away from the previously well-perfused segments. 17

What is TTE in pulmonary hypertension?

Transthoracic echocardiography (TTE) with Doppler imaging typically provides initial evidence supporting a diagnosis of pulmonary hypertension. Echocardiographic findings include right ventricular dilatation, hypertrophy, and hypokinesis, tricuspid regurgitation (TR), as well as pressure overload as evidenced by interventricular septal deviation toward the left. 8 Assessment of the TR jet allows reasonable quantification of the pulmonary arterial systolic pressure. Although sensitive for the detection of pulmonary hypertension, TTE does little to differentiate between the potential underlying etiologies in the absence of proximal pulmonary arterial thrombus, which is rarely seen. 9

What is CTEPH in pulmonary artery?

Chronic thromboembolic pulmonary hypertension (CTEPH) is defined as a MPAP greater than 25 mmHg that persists six months following diagnosis of pulmonary embolism. The prevalence of this disorder among the pulmonary artery hypertension population is unknown. CTEPH complicates acute pulmonary embolism in two to four percent of cases. 1,2 CTEPH is thought to be vastly under-diagnosed due to the fact that many patients do not have an obvious history of pulmonary embolism. 3 It remains unclear why some patients develop CTEPH following acute pulmonary embolism and others do not. Observational studies suggest an increased risk in patients with certain hypercoagulable risk factors and chronic medical conditions. 4,5,6 Pulmonary hypertension ultimately occurs as a result of macrovascular obstruction, small-vessel arteriopathy, and persistent vasoconstriction. 7

What is the gold standard for CTEPH?

Pulmonary angiography and right heart catheterization are the gold standard in the diagnosis of CTEPH and the assessment of operability. Angiography reliably differentiates chronic thromboembolic disease from the thrombus of acute pulmonary embolism. Angiographic patterns associated with chronic thromboembolic disease include vascular webs or bands, intimal irregularities, abrupt narrowing of the main pulmonary arteries, and obstruction of lobar or segmental vessels at their origin. 11

What is a V/Q scan?

Ventilation-perfusion (V/Q) scanning is an effective test to differentiate chronic thromboembolism from other potential etiologies of pulmonary hypertension. A normal V/Q scan reliably excludes CTEPH. 10 In a patient with pulmonary hypertension, multiple mismatched perfusion defects on V/Q scan are highly suggestive of chronic thromboembolism as the underlying etiology. Limitations of V/Q scanning include the inability to determine the exact location, burden, and extent of disease; which is vital information in formulating the appropriate treatment plan.

How to treat CTEPH?

The treatment of choice for CTEPH is pulmonary thromboendarterectomy. This procedure involves median sternotomy with cardiopulmonary bypass and periods of circulatory arrest during which the endothelialized clot is carefully dissected away from the underlying intima. Following successful thromboendarterectomy, patients immediately develop drastic reductions in pulmonary arterial pressure and pulmonary vascular resistance and improvement in cardiac output. 14 Hemodynamic improvements result in reverse right ventricular remodeling and return of normal right ventricular systolic and diastolic function. These physiologic changes result in significant improvements in functional capacity as evidenced by 6-minute walk distances and New York Heart Association (NYHA) class. 15 Benefits have been shown to persist for at least 9-12 months. Long-term mortality due to CTEPH following pulmonary endarterectomy is low among patients who survive three- months post-operatively; approximately one percent at one year and six percent at three years.

What is pulmonary angioscopy?

Pulmonary angioscopy involves the use of a fiberoptic scope for intraluminal pulmonary arterial visualization to the segmental level. Pulmonary angioscopy has largely been replaced by advancements in non-invasive imaging techniques. It still has potential value in operative planning for patients diagnosed with CTEPH; however, it would not be the initial test of choice.

What is a PAH?

Pulmonary arterial hypertension (PAH) is a rare , life-threatening disease defined by chronically elevated pressure in the pulmonary arteries [ 1 ]. The etiology of PAH can be idiopathic, familial, or associated with underlying disease, such as connective tissue diseases, certain autoimmune congenital cardiac conditions, and human immunodeficiency virus. PAH is also caused by exposure to drugs or toxins, namely anorectic agents and other stimulants [ 2 ].

What are the primary goals of PAH?

Primary treatment goals in PAH are to improve symptoms, stabilize or improve New York Heart Association functional class (NYHA FC), and prevent disease progression. Therapeutic agents act on one of the vasoactive pathways implicated in PAH. Endothelin-receptor antagonists (ERAs), phosphodiesterase-5 inhibitors (PDE-5is), ...