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Aortic surgery in a patient with erythropoietic protoporphyria

1 Department of Cardiothoracic Surgery, University Hospital of North Midlands, Stoke on Trent ST4 6QG, UK
2 Department of Cardiothoracic Anaesthesia, University Hospital of North Midlands, Stoke on Trent ST4 6QG, UK

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Date of Submission08-Feb-2022
Date of Decision20-Mar-2022
Date of Acceptance20-Mar-2022
Date of Web Publication11-May-2022


Erythropoietic protoporphyria (EPP) is the third most common porphyria caused by an inherited metabolic deficiency of porphyrin pathway. There is paucity of literature regarding conduct of anesthesia, complex cardiac surgery and postoperative management in these patients. We present a case of a 50-year-old patient presented with EPP who underwent aortic valve replacement and ascending aortic replacement successfully. We highlight the challenges in management of this condition in the perioperative period.

Keywords: Aortic surgery, cardiac surgery, erythropoietic protoporphyria, protoporphyria

How to cite this URL:
Srinivasan L, Meessala DK, Mishra PK, Jeeji R, Balacumaraswami L. Aortic surgery in a patient with erythropoietic protoporphyria. Ann Card Anaesth [Epub ahead of print] [cited 2022 Dec 3]. Available from:

   Introduction Top

Erythropoietic protoporphyria (EPP) is the third common porphyria, an inherited inborn error of porphyrin metabolism. It occurs due to partial deficiency (20% to 30%) of the enzyme ferrochelatase (FECH), the terminal enzyme of the hemobiosynthetic pathway needed to catalyze the insertion of iron in porphyrin molecule forming haem.[1] It is predominantly an autosomal dominant disorder though a recessive pattern of inheritance has also been demonstrated.[1],[2] In this condition, protoporphyrin accumulates in the skin, liver, and blood and is concentrated in the bone marrow, circulating erythrocytes, bile, and faeces causing skin photosensitivity, paraesthesia, and liver dysfunction.[1],[2]

A raised free erythrocyte protoporphyrin is measurable by red fluorescence microscopy at 620 nm in EPP,[1] but plasma protoporphyrin levels are not as high in EPP as in other porphyrias.[2] It absorbs light in the range of 320 to 595 nm (in blue spectrum) generating oxygen free radicals. Consequently, there is membrane lipid peroxidation, complement activation, and mast cell degranulation.[1],[2],[3] Vesicles develop on prolonged exposure to light and patients are advised to avoid sunlight and use sun-protective measures.[3]

The clinical manifestation is pruritis, burning, and pain followed by edema, erythema, urticaria, vesicular eruptions, crusting, and petechiae on exposure to light, as little as a few minutes.[1] The symptoms usually start in early infancy or childhood on first sun exposure, often lacking severe cutaneous signs.[3] Severe fatal hepatotoxicity occasionally seen in EPP is usually preceded by increased photosensitivity, erythrocyte, and plasma protoporphyrin levels.[2],[3],[4]

There is a paucity of literature on perioperative management of such patients undergoing major cardiac surgery. We report a 50-year-old EPP patient undergoing successful cardiac surgery for mixed aortic valve disease and ascending aortic dilatation. We highlight important measures taken during the perioperative period to achieve an optimal outcome.

   Case Report Top

A 50-year-old male with a body weight of 114 kg, suffering from EPP was referred with mixed aortic valve disease and dilated ascending aortic aneurysm 5.4 cm; sinotubular junction 4.6 cm and dilated sinus of Valsalva measuring 3.7 cm. Pre-operative biochemical tests including liver function tests were within normal limits. The patient was discussed in multidisciplinary team meeting to consider special requirements for this patient i.e anesthesia, procurement, and placement of special light filters and isolation room post-operatively.

Anesthesia was uneventful and induced with Midazolam, Fentanyl, and Propofol. Anesthesia was maintained with propofol and isoflurane during cardiopulmonary bypass (CPB) which lasted 3 hrs. Intra-operatively, trans-oesophageal echocardiography showed severe calcific pseudo- bicuspid aortic stenosis with the severely regurgitant fixed orifice. The patient underwent aortic valve replacement with 27 mm Sorin Bicarbon Fitline mechanical prosthesis and ascending aortic replacement with 28 mm Vascutek Gelweave graft. The patient underwent standard aorto-atrial bypass and was cooled down to 30.5° C. With a starting Hb of 123 gm/L, the pump was primed with crystalloid 1200 and 500 mls of colloid and hemoconcentration used to achieve a final Hb of 115 gm/L on separation.

Such complex cardiac surgery including anesthetic time, needing approximately 4-5 hrs under the direct theatre lights may trigger a photo-hypersensitive reaction. Hence, all the operating lights and windows were covered with Amber Plastic Madico (TA81) film (UVB filter) and entry to theatres was restricted to prevent inadvertent exposure. Porphyrin levels were monitored, and partial or total plasma exchange transfusions were considered but not necessary.

The patient was extubated for 4 hrs and post-operatively housed in a side room with the lights and windows covered with Madico (TA81) filter. He was discharged home on day 7 with normal blood including liver function tests. The hemoglobin was maintained above 100 gm/L post-operatively without any transfusion and the blood porphyrin level was raised to 19.1 μmol/L (normal 0.4 to 1.7) at discharge. He was mobilizing and wounds were healing well at discharge and 3-month follow-up clinic.

   Discussion Top

The prevalence of EPP ranges between 1:75,000 and 1:200,000 and does not have gender predominance.[1],[2] Patients with EPP undergoing major surgery need careful preoperative assessment and perioperative management of hemoglobin to minimize induction of porphyrin production.[1],[4]

The extent of damage from exposure depends on light quality i.e., wavelengths, duration of exposure, and clinical patient variables. Six out of 11 patients treated by liver transplantation developed photosensitivity causing burns to the abdominal wall, intestinal ulceration, and hemolytic anemia, and the death of two patients.[5] The exposure to operating theatre lights and risk of light-triggered failure in the severely compromised liver was not well recognized and cases in this series were done without any preventative measures like UV filters.[5]

Various filters have been evaluated and Amber/Orange plastic film Madico (TA81) was found to have a moderate influence on visibility, color perception with no significant restriction on working conditions.[3] On an experimental model, the hemolysis was minimal for about 12 hrs using Madico (TA81) filter compared to no filter.[3] In our case, we used the Madico (TA81) filter to achieve an optimal outcome. The choice of the filter is critical in cardiac surgery in order to be able to execute precise anastomosis i.e., coronaries. There are only three other reports of cardiac surgery in a patient with EPP and one of them is aortic valve replacement (AVR) and coronary artery bypass grafting (CABG) using Madico (TA81) filter.[6] The use of these filters poses challenges for the anesthetist who uses color-coded labels and the surgeon using blue prolene as blue objects are seen as black with the filter in the blue spectrum and other colors with a yellow tinge.

Drugs associated with precipitating porphyrin crisis like propofol, enflurane, ketamine, pancuronium, diazepam, etc. are best avoided or used cautiously in acute porphyria. Hypertension and tachycardia may be the only manifestations of porphyric crisis under general anesthesia and needs a high index of suspicion for early recognition. The skin changes, pain, nausea, vomiting, and neuropathy will not be detectable.[4],[7] Propranolol is recommended as it has additional benefits of attenuating the induction of hemsynthesis.[8] Intravenous haematin (Panhaematin, Abbot Laboratories, USA) and beta-blockers were made available in theatres to decrease haem synthesis in crisis but were not needed in our case as EPP is not associated with drug-induced crisis.[9]

Microcytic anemia is present in 20% to 60% of the patients with EPP.[2] Younger patients have preserved hemoglobin (Hb) levels and meticulous management of preoperative, intra-operative, and postoperative Hb is sufficient to maintain the Hb well above 100 gm/L. In the elderly age group with lower starting Hb, various techniques of Iron supplementation (oral/intravenous) and autologous blood donation (ABD) may be needed. Crystalloid and colloid prime leads to haemodilution and a drop in hemoglobin.[4],[6],[10] Besides, there is hemolysis during CPB made worse with the use of suction devices in the operative field.[6],[10] Usually, there is a requirement for autologous and/or allogenic blood transfusion in the perioperative period.[4]

We used ABD, smaller priming volume e.g., shorter length of CPB circuit; retrograde autologous priming, intraoperative cell salvage, and hemoconcentration on bypass. Attention was paid to meticulous surgical technique, and allogenic blood transfusion was avoided on CPB and postoperatively to prevent delayed hemolysis and/or liver derangement.

In conclusion, we report a rare case of a patient with EPP requiring complex cardiac surgery managed successfully. The unique challenges presented in the perioperative period can be managed by a meticulous multidisciplinary strategy.

Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent forms. In the form the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.

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Conflicts of interest

There are no conflicts of interest.

   References Top

Desnick RJ, Balwani M, Anderson KE. Inherited porphyrias. In: Pyertz RE, Korf BR, Grody WW, editors. Emery and Rimoin's Principles and Practice of Medical Genetics and Genomics: Metabolic Disorders. 7th ed. Academic Press; 2021. p. 373-411.  Back to cited text no. 1
Lecha M, Puy H, Deybach JC. Erythropoietic protoporphyria. Orphanet J Rare Dis 2009;4:19.  Back to cited text no. 2
Wahlin S, Srikanthan N, Hamre B, Harper P, Brun A. Protection from phototoxic injury during surgery and endoscopy in erythropoietic protoporphyria. Liver Transpl 2008;14:1340-6.  Back to cited text no. 3
Asokumar B, Kierney C, James TW, Amato J, Tuman KJ. Anaesthetic management of a patient with erythropoietic protoporphyria for ventricular septal defect closure. Paediatr Anaesth 1999;9:356-8.  Back to cited text no. 4
De Torres I, Demetris AJ, Randhava PS. Recurrent hepatic allograft injury in erythropoeitic protoporphyria. Transplantation 1996;61:1412-3.  Back to cited text no. 5
Akinci E, Erentug V, Bruzbuga N, Polat A, Mansuroglu D, Yakut C. Combined valve and coronary surgery in a patient with erythropoeitic protoporphyria. J Card Surg 2005;20:267-8.  Back to cited text no. 6
Jensen NF, Fiddler DS, Strieppe V. Anaesthetic considerations in porphyria. Anaesth Analg 1995;80:591-9.  Back to cited text no. 7
Disler PB, Eales L. The acute attack of porphyria. S Afr Med J 1982;61:82-4.  Back to cited text no. 8
Bissel DM. Treatment of acute hepatic porphyria with haematin. J Hepatol 1988;6:1-7.  Back to cited text no. 9
Yotsumoto G, Masuda H, Iguro Y, Kinjo T, Matsumoto H, Sakata R. Aortic valve replacement in a patient with erythropoietic protoporphyria. Ann Thorac Surg 2003;75:1003-5.  Back to cited text no. 10

Correspondence Address:
Pankaj K Mishra,
Consultant Cardiothoracic Surgeon, University Hospital of North Midlands, Stoke on Trent ST4 6QG
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/aca.aca_30_22