Case Report
Off-Pump Coronary Revascularization Using Bilateral Internal Thoracic Arteries in a Patient with Paroxismal Nocturnal Hemoglobinuria
Mariano Vrancic, Manuel Roque Cervetti*, Julián Benavides and Daniel Navia
Department of Cardiac Surgery, Instituto Cardiovascular de Buenos Aires, Olazabal 2706, P.C: 1428, Buenos Aires, Argentina
*Corresponding author: Manuel Roque Cervetti, Department of Cardiac Surgery, Instituto Cardiovascular de Buenos Aires, Olazabal 2706, P.C: 1428, Buenos Aires, Argentina
Published: 11 Feb, 2018
Cite this article as: Vrancic M, Cervetti MR, Benavides
J, Navia D. Off-Pump Coronary
Revascularization Using Bilateral
Internal Thoracic Arteries in a
Patient with Paroxismal Nocturnal
Hemoglobinuria. Ann Clin Case Rep.
2018; 3: 1503.
Abstract
Paroxysmal Nocturnal Haemoglobinuria (PNH), an ultra-orphan disease with a prevalence of
15.9 per million in Europe [1]. We report the first case in the literature of Off-Pump Coronary
Revascularization Using Bilateral Internal Thoracic Arteries in a patient with paroxismal nocturnal
hemoglobinuria.
A 36-year-old man presented to the emergency departmentfor acute non- ST elevation myocardial
infarction (NSTEMI).The patient presented paroxismal nocturnal hemoglobinuria diagnosed in
2016. Coronary angiography revealed a moderate stenosesostial and proximal in the left anterior
descending (LAD) coronary artery. There were chronicle stenoses of the left circumflex coronary
artery (LCX) and injury of 95% of the RCA. The conduits used for coronary revascularization were
both internal thoracic arteries (left ITA–right ITA [LITA-RITA]). We consider that OPCABG
using BITA can be safely performed with low in-hospital mortality and complications rates, even in
patient with PNH.
Keywords: Off-pump coronary revascularization; Bilateral internal thoracic arteries; Paroxismal
nocturnal hemoglobinuria
Introduction
Paroxysmal Nocturnal Haemoglobinuria (PNH), an ultra-orphan disease with a prevalence
of 15.9 per million in Europe, is a life-threatening disorder, characterized by haemolysis, bone
marrow failure and thrombosis [1]. PNH is based on a clonal defect of hematopoietic stem cells
characterized by deficiency in glycosyl-phosphatidylinositol (GPI)-anchored surface proteins due
to mutations within the X-chromosomal PIG-Agene [2]. PNH is an acquired hemolytic anemia
associated with an increased risk to develop thrombocytopenia, atypical venous thrombosis and
hypo plastic bone marrow [2].
We report the first case in the literature of Off-Pump Coronary Revascularization using Bilateral
Internal Thoracic Arteries in a patient with paroxismal nocturnal hemoglobinuria.
Case Report
A 36-year-old man presented to the emergency department for acute non- ST elevation myocardial infarction (NSTEMI) with elevation of troponins. Previous medical history included dilated cardiomyopathy with dyspnea funtional class II-III in study and coronary disease of three vessels. In addition, the patient presented paroxismal nocturnal hemoglobinuria diagnosed in 2016 and treated with Eculizumab. On admission, laboratory tests exhibited normal kidney function (blood urea nitrogen of 29mg/dl and creatinin of 0.93 mg/dl) and normal liver function. Leucocytes 4690/mm3. Differential blood count revealed 53.7% neutrophils, 33.7% lymphocytes, 11.1% monocytes, and 0.9% eosinophils. Hemoglobin 10.1 g/dl, Platelets 113.000/mm3. Coronary angiography revealed a moderate stenosesostial and proximal in the left anterior descending (LAD) coronary artery. There were chronicle stenoses in the proximal segment of the left circumflex coronary artery (LCX) and injury of 95 % in the third proximal of the RCA. Echocardiography showed reduced left ventricular function (Ejection fraction: 32 %), severely dilated left ventricle and global hypokinesia. The conduits used for coronary revascularization were both internal thoracic arteries (left ITA–right ITA [LITA-RITA]). The left internal mammary artery was harvested and anastomosed to the left anterior descending artery. The technical configuration was in-situ anastomoses of the LITA to the left anterior descending artery; and the RITA, after being divided at its origin and bifurcation, was connected end to side to the in-situ LITA as a sequential T graft to two circumflex arteries. Saphenous vein grafts were anastomosed to the posterior descending coronary artery. It was reported that skeletonized harvesting of ITA offer more conduit length and was associated with a lower incidence of sternal infection so we use this technic routinely. The procedure was off-pump without complication. The patient was transferred to the ICU after surgery. During the postoperative subcutaneous heparin 5,000 units was administered 2 times daily to avoid thromboembolism. Eculizumab (900 mg) was administrated during the postoperative to optimize hematological parameters.
Discussion
Thromboembolism is the most common cause of mortality in
PNH, and the 4-year survival of patients presenting with thrombosis
at diagnosis was only 40% before the era of eculizumab [3].
Eculizumab is a humanized monoclonal antibody that blocks terminal
complement pathway by binding to C5. This drug has dramatically
changed the natural history of PNH. Eculizumab increases transfusion
independency, reduces the risk of further thrombotic events and also
health-related quality of life. [4].
Cardiac surgery in PNH patients is associated with several
possible complications. PNH-induced granulocytopenia increases
the risk of postoperative infection. The aggravation of hemolysis by
extracorporeal circulation in cardiac surgery due to complement
activation from either contact of blood with the foreign material
surfaces during cardiopulmonary bypass circuit, or use of protamine
to neutralize systemic heparin after cardiopulmonary bypass and
tissue injury is well known [2]. We consider that OPCABG using
BITA can be safely performed with low in-hospital mortality and
complications rates, even in patient with PNH. Surgical tricks and
the new technology in coronary stabilizers allow surgeons to perform
a complete myocardial coronary revascularization using the best
available arterial conduit (BITA) [5]. There have been 5 cases reported
previously of patients with PNH undergoing cardiac surgery, but
this is the first case in where we combine Off-Pump Coronary
Revascularization Using Bilateral Internal Thoracic Arteries in a
patient with paroxismal nocturnal hemoglobinuria.
References
- Morag G, Talha M. Management of thrombosis in paroxysmal nocturnal hemoglobinuria: a clinician’s guide. Ther Adv Hematol. 2017;8:119-126.
- Knobloch K, Lichtenberg A, Leyh RG, Schubert J. Aortic valve replacement and coronary revascularization in paroxysmal nocturnal hemoglobinuria. Interact Cardiovasc Thorac Surg. 2003;2:647-649
- Quinquenel A, Maestraggi Q, Lecoq-Lafon C, LatourRégis P, Delmer A, Servettaz A. Atypical presentation of paroxysmal nocturnal hemoglobinuria treated by eculizumab: A case report. Medicine. 2017; 96:12.
- Hillmen P, Muus P, Roth A, Elebute MO, Risitano AM, Schrezenmeier H ,et al. Long-term safety and efficacy of sustained eculizumab treatment in patients with paroxysmal nocturnal haemoglobinuria. Br J Haematol. 2013;162:62-73.
- Navia D, Vrancic M, Vaccarino G, Piccinini F, Raich H, Florit S, et al. Total arterial off-pump coronary revascularization using bilateral internal thoracic arteries in triple vessel disease: Surgical technique and clinical outcomes. Ann Thoracic Surg. 2008;86:524-531.