Received Date: November 03, 2021; Accepted Date: November 15, 2021; Published Date: November 22, 2021
Citation: Bafna AA, Deokate V, Bagani P, Prasad S, Mulla M, et al (2021) Acute Lower Limb Ischemia as Presenting Symptom in Case of Infra Renal Coarctation of Aorta: A Rarity. Interv Cardiol J Vol.7 No.11:158
Copyright: © 2021 Bafna AA, et al. This is an open-access article distributed under the terms of the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original author and source are credited.
We describe a case of infra-renal coarctation of aorta in a 40-yr-old woman who presented with pain and black discoloration of third small toe of left lower limb. She had neither past medical history nor associated coronary artery disease risk factors. Computed tomographic angiography showed a narrowing of infra-renal aortic segment, with monophasic blood flow in left lower limb arteries, bilaterally. After a coarctoplasty, triphasic pulse waveform restored in arteries of left lower limb bilaterally, and clinical improvement was observed in pain and blackish discoloration. To our best knowledge, we first to describe the case of infra-renal coarctation of aorta presented with left lower limb acute ischemia.
Acute ischemia; Aorta; Coarctoplasty; Infra-renal coarctation; Middle aortic coarctation (MAC); Toe
Middle aortic coarctation (MAC) or abdominal aortic stenosisis an extremely rare form vascular anomaly with almost 200 cases reported in literature . On the basis of location, it is further classified as supra-renal, inter-renal and infra-renal coarctation of aorta. Hypertension proximal to the aortic coarctation and comparative hypotension distal to it are characteristic investigative finding in MAC. The clinical manifestations of MAC include headache, fatigue on slight exertion and bilateral lower-limb claudication . By the best of our knowledge on the available literature, we arepresenting first-time the case of infrarenal coarctation of aorta presented with left lower limb acute ischemia.
A 40-year-old female presented to Diamond Hospital, Kolhapur, and Maharashtra, India on 13 August 2021 with a complaint of black discoloration of thirdsmall toe of left lower limb from 2 days and pain from 3-4 days (Figure 1A). She had no past medical history with any coronary artery disease risk factors. She reported two successful and uneventful pregnancies. Her blood pressure was measured as 149/96 mm of Hg with pulse rate of 120/minute on examination. There were weak pulses noted bilaterally in left lower limb; however, rest of the other physical examination and pulsation were within normal limit. The routine blood investigations, including ANA blot test, were within normal range and ruled out autoimmune disorders. Electrocardiography revealed sinus tachycardia with no other abnormality. The 2D echocardiography reported Left ventricular ejection fraction (LVEF) 60%, and other structures as well dimensional parameters were within normal limit. A Doppler ultrasonography of abdomen and lower limb revealed a significant narrowing of abdominal aorta. There was monophasic blood flow in left lower limb arteries, bilaterally, with gradient across the coarctation measured as 86 mm of Hg. A computed tomography angiogram reported a narrow aortic segment of 18.6 mm in length present below the origin of renal arteries. The diameter of aorta at diaphragm was 16.0 mm, whereas pre and post-coarctation werenoted as 18.2 mm and 16.7 mm, respectively (Figure 2A). The tightness of the coarctation segment was reported as 6.0 mm (Figure 2A).These findings suggested for the diagnosis of infra-renal coarctation of aorta. Preoperative pharmacological management included Aspirin 75 mg (OD); Clopidogrel 75 mg (OD); Atorvastatin 80 mg (HS); Rivaroxaban 2.5 mg (BID); Pentoxafyllin 40 mg (TID); cilostazol 50 mg (BID); Telmisartan 40 mg (HS) and Amlodipine 5mg (HS) for optimal reperfusion and blood pressure control.
The coarctoplasty was performed with consultation of cardiovascular team, using ANDRA-Med balloon (14mm x 4 cm), and ANDRA-MEDAS XL-43 (16mm x 4cm) stent was inserted successfully and uneventfully after preserving bilateral renal arteries (Figure 2B-E). Post coarctoplasty with stenting, the pressure gradient across coarctation reduced from 86 mmHg to 12 mmHg.
The patient had an uncomplicated postoperative course. Postoperative management included Aspirin 75 mg (OD); Atorvastatin 80 mg (HS); Rivaroxaban 2.5 mg (BID); Telmisartan 40 mg (HS) and Amlodipine 5mg (HS). On day three postoperatively, the pulses were noted normal in the left lower limb. The triphasic pulse waveform restored in arteries of left lower limb bilaterally was confirmed with Doppler ultrasonography (Figure 2F). The gradient at the site of coarctation reduced to 12 mm Hg postoperatively. A clinical improvement was observed in pain and blackish discoloration (Figure 1B).
Figure 2: Computed tomography angiography of the abdominal aorta and both lower extremities of patient, before and after procedure showing A, severe stenosis of abdominal aorta just below to origin of the renal arteries with pre and poststenotic dilation associated with the evidence of infra-renal aortic stenosis; B-D, evidence of coarctoplasty using ANDRA-Med balloon (14 mm x 4 cm); E-F, evidence of an implantedANDRA-MEDAS XL-43 (16mm x 4cm) stent proximal to the renal arteries with a good patency of abdominal aorta, ciliac artery, superior mesenteric artery, and renal arteries
We demonstrated a rare case of infra-renal coarctation of the aorta presented with acute left lower limb ischemia manifested as pain and black discoloration of the third small toe.
Middle aortic coarctation (MAC) is an extremely rare vascular anomaly with an occurrence rate of almost 0.002%. . Though the exact etiology of MAC is unknown, proposed causes of MAC are congenital anomalous development, rubella infection or syndrome; obliterative pancreatitis, and fibromuscular dysplasias .
MAC has been classified as type I, suprarenal coarctation with renal artery stenosis; type II, infrarenal coarctation with renal artery stenosis; type III, suprarenal coarctation with normal renal arteries; and type IV, infrarenal coarctation with normal renal arteries . In our case, infra-renal coarctation of the aorta presented with normal renal arteries. However, on presentation, her blood pressure was noted high (140/96 mmHg) than normal with a pulse rate of 120/minute. There was no cardiovascular or another medical history; perhaps this increased blood pressure might be due to infra-renal coarctation of the aorta in this case.
The involvement of other arteries stenosis is also common with MAC . Surprisingly, there was no other arterial stenosis observed in our patient. Our patient had none of the investigative findings associated with the etiologic diseases or factors. The routine blood investigations, including the ANA blot test, were within normal range and ruled out autoimmune disorders in the present case. Thus, we demonstrated a case with a rare type of infrarenal abdominal aortic coarctation presented with acute lower limb ischemia.
We reported the first case of infra-renal coarctation of aorta presented with left lower limb acute ischemia which is a very rare finding. Infra-renal coarctation of aorta or MAC may lead to lower limb acute ischemia and tissue necrosis and may be presented with painful and discoloration of tissue.