Kussmaul’s Sign- In a patient with Severe PAH

Abstract

The patient is a 33 years old male who had a history of acute pulmonary embolism three and a half years back. He had received thrombolysis with alteplase and was subsequently on oral anticoagulants. After one year of the index episode, the patient started to experience progressive worsening of breathlessness. Further evaluation led to the diagnosis of chronic thromboembolic pulmonary hypertension (CTEPH). Patient was offered pulmonary endarterectomy which he declined and preferred medical management. He did not show clinical improvement and eventually developed refractory heart failure. And patient presented to Emergency department (ED) with worsening of breathlessness. On presentation to the ED, patient was in distress with pulse of 110/min, BP of 100/60 with no evidence of pulsus paradoxus, Respiratory rate of 22/min and chest was revealing bilateral basal crepts and CVS examination revealed pansystolic mummer at left parasternal edge. JVP was elevated and showed only one prominent outward crest, which is a prominent CV wave and one dominant downward trough, a prominent Y descent, and there was a paradoxical rise in the JVP on inspiration (video 1) which is an important clinical sign in heart failure commonly known by the eponym Kussmaul’s sign. The prominent CV wave in this paitent reflected sever Tricuspid regurigitation (TR). On echo, patient had severe RV dysfunction with severe TR with Pulmonary hypertension. Patient was treated with intravenous diuretics, and pulmonary vasodilators and improved symptomatically and was referred for work up for heart-lung transplantation.