Clinical Cases in Chronic Thromboembolic Pulmonary Hypertension

This practical casebook provides important insight to clinicians looking to learn about the diagnosis and management of chronic thromboembolic pulmonary hypertension (CTEPH). It systematically describes the clinical decision-making process for a variety of CTEPH cases, from common to rarely seen examples of this disease. Chapters are constructed in an easy-to-follow format designed to improve the reader’s clinical decision-making.

Clinical Cases in Chronic Thromboembolic Pulmonary Hypertension features not only a number of CTEPH cases of varying complexity, but also several medical conditions that may mimic CTEPH. It is therefore a vital evidence-based resource for trainee and practising internists, cardiologists, pulmonologists, cardiac surgeons and radiologists looking to expand their knowledge in this area.

• Language: English
• Publisher: Springer
• Release date: Sep 30, 2019
• ISBN: 9783030173661

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© Springer Nature Switzerland AG 2020

W. R. Auger, D. Gopalan (eds.)Clinical Cases in Chronic Thromboembolic Pulmonary HypertensionClinical Cases in Cardiologyhttps://doi.org/10.1007/978-3-030-17366-1_1

1. Case 1: The Evaluation of Chronic Thromboembolic Pulmonary Hypertension

Manreet Kanwar¹  , Deepa Gopalan²   and William R. Auger³  

(1)

Cardiovascular Institute at Allegheny Health Network, Pittsburgh, PA, USA

(2)

Department of Radiology, Imperial College Hospitals, London, UK

(3)

Lewis Katz School of Medicine, Temple University, Philadelphia, PA, USA

Manreet Kanwar (Corresponding author)

Email: [e-mail]

Deepa Gopalan

Email: [e-mail]

William R. Auger

Email: [e-mail]

Keywords

Chronic thromboembolic pulmonary hypertensionComputed tomography angiographyLung scintigraphyCatheter-based pulmonary angiography

Case Presentation

Patient is a 68-year-old African American male who was referred to us for evaluation of worsening shortness of breath in the setting of previous history of pulmonary emboli (PE) and recently diagnosed pulmonary hypertension (PH). He was initially diagnosed with unprovoked bilateral deep venous thrombi (DVT) and PE in 2013. Hypercoagulability work-up was negative. He was started on warfarin for anticoagulation which was subsequently discontinued after a year. He again presented in June 2016 with worsening shortness of breath from a recurrent PE. Anticoagulation was resumed with apixaban with some initial improvement in his symptoms. An IVC filter was placed (although there was no documented recurrent DVT). The patient reported increasing shortness of breath over the following months, with associated lower extremity swelling and abdominal distention. He was evaluated as an outpatient and started on diuretics and treated for bronchitis for several weeks. His symptoms eventually started affecting his activities of daily living when he presented to an outside hospital for evaluation in September 2016, after an event of near syncope following moderate exertion.

His initial chest radiograph (Fig. 1.1) and lab work were unremarkable for any acute findings.

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Figure 1.1

Chest radiograph upon presentation

An echocardiogram showed severe right ventricular dilatation (RVEDD 57 mm) with moderately reduced right ventricular global systolic function (TAPSE 11 mm) and mild hypertrophy (Fig. 1.2). His left ventricular ejection fraction was preserved at 65% and his calculated pulmonary artery systolic pressure (PASP) was about 105 mmHg, with a small, circumferential pericardial effusion.

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Figure 1.2

TTE pre-op (parasternal long-axis view, four-chamber view; TR jet). The right ventricle is severely dilated with moderately to severely reduced systolic function. There is moderate hypertrophy of the right ventricle. The right ventricular systolic pressure is 101 mmHg

Given his previous history of PEs, he underwent a repeat CT angiogram of his chest which showed chronic pulmonary emboli bilaterally (Fig. 1.3). The largest thrombus burden was at the distal portion of the right pulmonary artery with extension and occlusion of the right lower lobe interlobar pulmonary artery as well as the proximal right middle lobe pulmonary artery. At that point he was started on diuretics, placed on 5 L oxygen via nasal cannula and transferred to our institution for further management.

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Figure 1.3

CT angiography of the chest with findings diagnostic of CTEPH. Enlarged bronchial collateral artery: solid white arrow

His past medical history was also significant for ongoing smoking with over 20 pack year history of tobacco abuse. He denied any history suggestive of connective tissue disease, liver or kidney disease, malignancy, thyroid or hematological disorders, or other risk factors for pulmonary hypertension.

His EKG showed right axis deviation with borderline right ventricular hypertrophy in a sinus rhythm. V/Q perfusion images revealed large mismatched defects bilaterally (Fig. 1.4).

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Figure 1.4

Preoperative lung perfusion scintigraphy

Right heart catheterization showed significantly elevated right-sided filling pressures with a right atrial (RA) pressure of 20 mmHg, a PAP of 71/33 mmHg (mean 45) and a pulmonary capillary wedge pressure of 11 mmHg. His cardiac index was marginal at 2.2 L/min/m² and his calculated pulmonary vascular resistance (PVR) was 9.1 Wood Units. Pulmonary angiogram showed lack of perfusion in his right lower and middle lobes with multiple other findings consistent with CTEPH (Fig. 1.5a, b). Coronary angiogram revealed no significant obstructive disease.

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Figure 1.5

(a, b) Catheter-based pulmonary angiography illustrating features of chronic thromboembolic disease. Angiographic web depicted by solid white arrow

His pulmonary function tests showed moderate obstructive lung disease with an FEV1 of 2.1 L.

He was admitted for aggressive diuresis, and given his hemodynamic presentation we used inotropic support with IV milrinone for 72 h prior to surgery for volume and right ventricular optimization. After obtaining informed consent, he underwent pulmonary thromboendarterectomy (PTE) surgery with the right side circulatory arrest time of 32 and 16 min on the left side (Fig. 1.6).

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Figure 1.6

Clot specimen removed at the time of pulmonary thromboendarterectomy

He was extubated the following morning and weaned off of his inotropic support over the next 3 days. Off support, his postoperative PA pressures were RA 6 mmHg, PA 33/9 mmHg (mean 19), PCWP 7 mmHg, CI 2.7 L/min/m², PVR 2.7 Wood Units, BP 107/82. He was discharged home 7 days later and had a follow-up right heart catheterization 6 months later, which demonstrated a mean PAP of 23 and a PVR of 2.8 Wood Units. He continues to do well at his 1 year