Surgical treatment and endarterectomy must be bilateral because this is a bilateral disease in the vast majority of patients. The procedure is conducted with the aid of cardiopulmonary bypass, and the patient is cooled to allow for hypothermic circulatory arrest. Meticulous postoperative management is essential to the success of this surgical procedure.
All patients are mechanically ventilated for at least 24 hours, and all patients are subjected to maintained diuresis. Patients are subject to all complications associated with open-heart and major lung surgery (eg, arrhythmias, atelectasis, wound infection, pneumonia, renal failure, stroke, mediastinal bleeding) but may also develop complications specific to this operation. These include persistent pulmonary hypertension, reperfusion injuries, and neurologic disorders related to hypothermic arrest and deep hypothermia. Reduction in pulmonary pressures, resistance to normal levels, and corresponding improvements in pulmonary blood flow and cardiac output are generally immediate and sustained. In general, these changes can be assumed to be permanent.
Prior to surgery, more than 95% of the patients are in New York Heart Association (NYHA) functional class III or IV. After surgery, 95% of patients remain in NYHA functional class I or II after 1 year. After successful surgery, reduction in pulmonary vascular resistance exceeding 50% is usually seen and translates into improved functional status of the patient, with 6-year postsurgery survival of 75%. After surgery, 93% were in NYHA class I or II, compared with about 95% who were NYHA class II or IV preoperatively.
Medical therapy for patients with CTEPH should be considered in patients in whom PTE is contraindicated because of significant comorbidities or unresectable (distal) chronic thrombus, and in patients who elect not to undergo PTE by personal choice. Also, medical therapy is beneficial as a “stabilizing bridge” to surgery to reduce the postoperative mortality risk in patients who have severe pulmonary hypertension and/or right heart failure, and in patients who have residual pulmonary hypertension after surgery. Pulmonary vasodilator therapies used in idiopathic pulmonary arterial hypertension (IPAH) have been evaluated in patients with CTEPH, including prostacyclin analogs, endothelin receptor antagonists, and phosphodiesterase-5 inhibitors. Soluble guanylate cyclase (sGC) stimulators have been approved by US Food and Drug Administration (FDA) for the treatment of IPAH and CTEPH. Lifelong anticoagulation therapy is recommended for all patients with CTEPH, but it does not generally affect the underlying disease process of CTEPH. Lung transplantation can be considered for patients who do not respond to medical therapy or in those whose disease is not amenable to correction by PTE.
CTEPH is one of the leading treatable causes of pulmonary hypertension. This case emphasizes the need for clinicians to be aware of CTEPH because its diagnosis is commonly delayed or missed because of the nonspecific nature of its presentation and, sometimes, because of a lack of prior history of acute symptomatic PE or deep vein thrombosis. All clinicians should have a high index of suspicion and consider pulmonary vascular disease (thromboembolic disease/ pulmonary hypertension) in the differential diagnosis of any patient complaining of unexplained persistent dyspnea.
The patient in this case was admitted and started on anticoagulation (heparin and warfarin) and an inferior vena cava filter was placed. He was evaluated by a thoracic surgeon with expertise in CTEPH and offered PTE, but he refused. He was also evaluated by a pulmonary hypertension specialist who started him on intravenous epoprostenol and bosentan for his elevated pulmonary pressures. After achieving a therapeutic international normalized ratio, the patient was discharged to follow-up with the pulmonary hypertension clinic. After 2 months of medical therapy, the patient's dyspnea moderately improved.
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