Case report:
A 20 years old male, a known case of SCD, with history of stroke with minimal right-side weakness. He was diagnosed with aortic valve stenosis under conservative management. The patient was referred to our center as a case of acute infective endocarditis, bicuspid aortic valve with sub aortic membrane stenosis. His main complaint was fever otherwise no other symptoms (no chest pain, no shortness of breath, no nausea, vomiting or change in bowel habits, no history of contact with an ill person or traveling, no contact with animal or raw milk ingestion)
On examination: Patient was conscious, oriented and hemodynamically stable. Chest was clear on auscultation, with normal oxygen saturation on room air. The patient was febrile with temperature > 38C.
Precordial auscultation showed harsh ejection systolic murmur with crescendo-decrescendo configuration, medium pitch with grade III intensity, best heard over the aortic area radiated to carotid.
Blood test, showed Hb of 7.4 g/dl, HBs 92.2, WBC of 21, three blood cultures from different sites were negative. Q-fever, Brucella and Dengue fever workup were negative. The patient was started on empirical IV antibiotics ceftriaxone and vancomycin.
Trans-thoracic echocardiography showed severe concentric hypertrophy, normal left ventricular (LV) function with LV ejection fraction of 60%. Aortic valve (AV) was bicuspid with no evidence of vegetation, ascending aortic root dilation 4.1 cm. Trans-esophageal echocardiographyshowed thickened and restricted AV leaflets, there was severe aortic valve stenosis with peak and mean gradients of 160 and 90 mmHg respectively. There was no vegetation or aortic root abscesses. (Figure 1 a, b, c).
CT chest and abdomen: was done looking for a source of infection, it showed generalized lymph node enlargement in the axilla, abdomen, and inguinal area. An excisional biopsy of left inguinal LN (3*2 cm) was sent for histopathology and showed Hodgkin lymphoma (Figure 3).
Brain CT + CT Angiogram: Re-demonstration of multiple old infractions mainly seen in the occipital lobe, posterior limb of the right internal capsule and the corona radiata/centrum semiovale with encephalomalacia changes. No evidence of acute infraction. No acute hemorrhage (Figure 2). CT angiography was unremarkable.
MRI: was done and showed multiple old infarctions predominantly in the occipital region and the corona radiata/centrum.
PET scan (was done after the surgery): Multiple FDG avid enlarged lymph nodes seen in the left inguinal, obturator and external iliac lymph node chain. The largest measures around 3.2 cm (SUV max 15). The urinary bladder demonstrates irregular outlines with trabeculation (Figure 4). Evidence of FDG avid adenopathy in the pelvis, as detailed above in keeping with history of lymphoma. Post-operative activity in the mediastinum and myocardium/pericardium, for clinical correlation. Abnormal appearance of the urinary bladder for clinical correlation.
The patient was scheduled for AV and ascending aorta replacement.
Cardiopulmonary bypass (CPB) management: The decision was made to perform an exchange transfusion using packed red blood cells (PRBCs) in the pre-operative period and immediately before initiation of CPB.
The patient’s height was 160 cm and weight 40 kg, body surface area of 1.33 m2 and his hemoglobin level was 6.35g/L.
The circuit was adapted for the exchange transfusion by adding a 1/4-3/8-3/8″ Y connector within the venous line. The two 3/8″ ends of the Y were connected to the standard venous line of the circuit. The 1/4″ end of the Y was connected to 1/4″ tubing and attached to a separate cardiotomy reservoir (cell saver).
Circuit was primed with 900 ml of crystalloid fluid. After that the crystalloid fluid was replaced with 250 ml of 5%albumen, 50 mEq of sodium bicarbonate, 10,000 units of heparin, 2g of cefazolin, and 100 ml of 5% mannitol. After the circuit was primed, the excess crystalloid fluid was drained from the circuit, and 4 units of PRBCs were added to the venous reservoir.
Once the chest was opened and the heart was exposed, the patient was systemically heparinized. An 18Fr aortic cannula was placed in the ascending aorta. Venous drainage was accomplished through straight cannulation with 32/40 Fr double stage venous cannula placed in the right atrium. Once an activated clotting time of 371 seconds was achieved, the exchange transfusion was initiated. With the venous line clamped distal to the Y connector, the line to the separate cardiotomy reservoir was opened, allowing the patient’s blood to drain. At the same time, blood from CPB machine was transfused to the patient to maintain the patient hemodynamically stable. After an exchange transfusion of 1500 mL was achieved, the patient was placed on CPB.
Normal hypothermia was used, and the patient was cooled to a bladder temperature of 34°C. Antegrade cardioplegia cannulas were placed. An aortic cross clamp was placed, and the heart was initially arrested with antegrade cold-crystalloid. A St. Jude mechanical aortic valve size 21-mm was implanted and ascending aorta was replaced with vaxcotec graft size 26mm. The CPB flows were maintained at a minimum cardiac index of 2.4 L/min. Venous saturation was kept >70% and no acidosis occurred during the CPB period. Two units of PRBCs were given during the bypass run. Hemoglobin level was 10.6g/L. The patient was re-warmed to a bladder temperature of 36°C. The aortic cross-clamp was removed after 134minutes. After resumption of normal sinus rhythm, the patient was ventilated and successfully weaned from CPB. Total CPB time was 163minutes. There were surgical oozing with no obvious site of bleeding, multiple rounds of blood products (FFP, PRBCs) were given and decision was made to leave the chest opened and transfer to the intensive care unit (ICU).
In the ICU, the patient required surgical re-exploration for hemostasis with no site for bleeding, and another round of blood product were given, and bleeding stopped. Chest was closed on the second postoperative day and the patient was extubated. (6)
All chest tubes were removed in timely fashion, his general condition continued to improve and he was discharged in a stable condition. Follow up appointments with oncology and hematology outpatients’ clinics were given to continue with management. In view of his early stage Hodgkin’s Lymphoma and absent B symptoms (fever, night sweats, and weight loss), involved site radiation therapy (ISRT) up to 36 Gy as a single Modality.
Recently, the patient was admitted through the ER as a case of acute right occipital ischemic stroke. Regarding cardiac point of view, there were no complaints and he showed a significant improvement in the symptoms. There was no major surgical complication. The admission duration was 2 weeks after which he was discharged in a stable condition.