Title:
Bilateral Upper Extremity Critical Limb Ischemia Following Lung Transplantation due to history of Scleroderma-Associated Interstitial Lung Disease
Introduction:
Scleroderma (systemic sclerosis) is a chronic autoimmune disease that affects connective tissues with a reported mortality rate of 50% at 5 years.1 Progressive systemic sclerosis is often accompanied with Raynaud’s phenomenon and can affect the lungs leading to Scleroderma-associated interstitial lung disease that may require a lung transplant.1-2 Patients who suffer from scleroderma with Raynaud’s phenomenon have been linked to develop accelerated atherosclerosis, increased cardiovascular morbidity, and critical limb ischemia (CLI).3 Literature regarding management of patients with progressive scleroderma and CLI have been limited.
Clinical Case:
A 49-year-old female with a past medical history of interstitial lung disease, scleroderma, and hyperlipidemia presented for a bilateral lung transplant. Post-transplant, the patient returned to the critical care unit with veno-venous extracorporeal membrane oxygenation support and with low dose vasopressin and norepinephrine drips. Within 24 hours post-procedure, the patient developed an acute loss of bilateral radial and ulnar arterial pulses.
The patient underwent a bilateral upper extremity arterial duplex that demonstrated no flow in bilateral distal ulnar arteries and mid-distal radial arteries with patent flow within the bilateral subclavian, axillary, and brachial arteries. During this time, the patient developed acute pain in bilateral hands and the dorsal surface of her distal upper extremities. The patient was then referred for a bilateral upper extremity angiogram. We obtained bilateral femoral access with a micro-puncture kit and ultrasound guidance with placement of two 6 French 11cm sheaths. We performed bilateral upper extremity angiograms using 5Fr Multipurpose catheters with side-holes. Both angiograms demonstrated small, patent subclavian, axillary, and brachial arteries with slow flow, and an obliteration of contrast past the proximal radial and ulnar arteries. This was thought to be due to microvascular spasm and no reflow phenomenon. We delivered 5mg of tissue-type plasminogen activator and 18mg of papaverine to help facilitate any breakdown of thrombus and to encourage vasodilatation respectively distally. We then placed bilateral Uni-fuse catheters in the upper extremities and started a papaverine drip at 15miligrams/hour for 12 hours.
During the papaverine infusion, the patient did have return of bilateral radial and ulnar pulses via doppler signal.
Discussion:
Papaverine is an opium alkaloid that provides the relaxation of vascular smooth muscle that affects both the coronary and peripheral circulation. Prior reports have described the use of selective intraarterial papaverine infusion for spastic vascular territories particularly in the cerebrum and for harvesting mammary arteries for bypass surgery. Papaverine has also been approved to treat erectile dysfunction and spasms of the gastrointestinal tract. However, there has been limited demonstrations of clinical efficacy of papaverine use in severe Raynaud’s phenomenon or in patients with progressive scleroderma in the setting of CLI.
In the literature, there is no mainstay treatment for patients with advanced scleroderma leading to a Raynaud’s crises or CLI. Our case supports endovascular management with the delivery of continuous papaverine infusion to help reestablish arterial flow and treat arterial vasospasm in patients with advanced scleroderma with CLI.