A GP contacts you about a young woman in whom a formal diagnosis of pulmonary hypertension has been made. He has not yet received her discharge summary but tells you that she has had a ‘range’ of tests.
Which one of the following would have to have been true in order for the diagnosis to be made?
Pulmonary hypertension (PH) is defined as mPAP ≥25 mmHg at rest using invasive measurements made during right heart catheterization. Values during exercise are currently excluded from this definition. In PH, a normal wedge pressure suggests that the cause is not left heart disease. A normal-sized right heart does not exclude PH. Conversely, the presence of pulmonary emboli does not mean that the pulmonary artery pressure will definitely be elevated.
You are asked by one of the echocardiography technicians to review an echocardiogram for an elderly patient who presents with breathlessness. Estimated RV systolic pressure, as judged by the velocity of the tricuspid regurgitant jet, is moderately elevated.
In terms of an aetiology of the pulmonary hypertension, which one of the following is true?
The echocardiogram in PH can be instructive as to the underlying aetiology. Left heart disease and lung disease/hypoxia are common causes. Left atrial dilatation is a red flag for left heart disease. Be mindful of systemic disease processes such as connective tissue disease which can underlie pulmonary arterial hypertension. Proximal pulmonary artery thrombus can form in situ due to sluggish flow when the pulmonary arteries are dilated, especially in patients with Eisenmenger syndrome.
You admit a middle-aged woman on the acute take who has been investigated for breathlessness for several years. Pulmonary hypertension is suspected.
What should you do?
Routine therapies given for patients with PH include warfarin (even if the underlying aetiology is not chronic thromboembolic disease) to attenuate the risk of in situ thrombus formation. Diuretics are given in those with overt right heart failure. Involvement by a specialist centre is essential. Advanced drug therapies should not be started without specialist input as they can be harmful. ACE inhibitors are not indicated for right heart failure.
A 70-year-old woman with a history of proven recurrent pulmonary emboli but no other comorbidities presents with breathlessness over a number of months. She is in NYHA class III. Her INR has been within the therapeutic range. Serial echocardiograms demonstrate persistent features of pulmonary hypertension.
Which one of the following is the most important measure?
This description fits with a diagnosis of chronic thromboembolic PH (CTEPH). In those patients with CTEPH in whom anticoagulation at therapeutic levels has proved ineffective at bringing down pulmonary pressures, consideration should be given to surgery with pulmonary end-arterectomy. This procedure can be curative in carefully selected patients. Advanced therapies can be considered if the patient is felt to be inoperable. Balloon atrial septostomy, which permits the right heart to vent into the left atrium, is used infrequently in selected patients with refractory syncope and heart failure.
You have been contacted by the infectious diseases team about a man with HIV who has become progressively more breathless over a series of months. He has had a CT pulmonary angiogram which has excluded clots. You discuss the scan with the radiologist.
What else would you like to know about the CT?
CT can provide important information in addition to the presence or absence of pulmonary emboli. An enlarged right ventricle (though it can be normally sized), right atrium, and pulmonary arteries are all features of PH. Contrast reflux into the inferior vena cava implies tricuspid regurgitation and increased right atrial pressure. The latter is also indicated when the right atrium is dilated. The position of the interventricular septum and the interatrial septum reflects the balance of pressures between the ventricles and atria, respectively.
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