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Case Report

Assessment of Carotid Body Tumor and Its Association with Tetralogy of Fallot: Effect of the Chronic

Banu Sahin Yildiz, Ahmet Sasmazel1, Ayse Baysal2, Hulya I. Gozu, Emre Erturk3, Ozge Altas1, Rahmi Zeybek1, Alparslan Sahin4, Mustafa Yildiz5 Departments of Internal Medicine, Dr. Lütfi Kırdar Kartal Educational and Research Hospital, Departments of 1Cardiovascular Surgery, 2Anesthesiology and Reanimation, and 3Cardiology, Kartal Koşuyolu Yuksek Ihtisas Educational and Research Hospital, 4Departments of Cardiology, Bakirkoy Dr. Sadi Konuk Educational and Research Hospital, and 5Departments of Cardiology, Istanbul University Cardiology Institute, Istanbul, Turkey

ABSTRACT

This is a rare combined presentation of Tetralogy of Fallot and carotid body tumor (CBT). Hypotheses and further discussion provides data for the development of CBT as a response to chronic hypoxemia. This present study demonstrates and discusses such an occurrence.

Key words: Carotid body, carotid tumor, hypoxia, Tetralogy of Fallot

How to cite this article: Yildiz BS, Sasmazel A, Baysal A, Gozu HI, Erturk E, Altas O, et al. Assessment of carotid body tumor and its association with tetralogy of fallot: Effect of the chronic hypoxia. Views 2014;15:86-8. © Gulf Heart Association 2014.

INTRODUCTION CASE REPORT

he carotid body is a organ buried A 20‑year‑old woman living at high posteriorly in the adventitia of the common carotid altitude (1700 m) was referred to our hospital with Tartery bifurcation.[1] It is originated from the cyanosis and a right mass. She was neither mesodermal elements of the third arch and the hypertensive nor diabetic and did not have any family ectoderm. The carotid body is involved with history of . A right neck mass had the chemoreceptor control of pressure, been discovered for 2 years. Physical examination and respiration.[1] It detects changes in the composition revealed a cyanotic woman without clubbing of of flowing through it, mainly the partial the fingers and toes. In the neck, there was about pressure of oxygen (PO2), but also of of 1.5 mm × 1.0 mm pulsatile mass. The jugular veins (PCO2). Furthermore, it is also sensitive were distended. Auscultation detected a harsh to changes in pH and temperature. grade III/VI midsystolic murmur at the left upper are uncommon neoplasms of the sternal border that did not radiate to the neck. The head and neck that arise from the neural crest cells.[2] liver was slightly enlarged. Histopathologically similar to the pheochromocytoma; The laboratory data included; hematocrit: 50%, it is usually benign and non‑functional. Carotid body PO2: 75 mmHg, PCO2: 35 mmHg, pH: 7.42 (pH; minus tumor is the most common form of paragangliomas of the decimal logarithm of the hydrogen ion activity). The the head and neck area.[2] The association of carotid highest PO2 recorded was 83 mmHg. The erythrocyte body tumors (CBTs) with high altitude habitation sedimentation rate was 27 mm/h. Glucose, urea, [3‑5] and chronic hypoxemia has been documented. creatinine, electrolytes, thyroid function tests with Recent literature has proposed the development of CBTs as a response to the chronic hypoxia in Access this article online [4,5] patients with cyanotic congenital heart disease. Quick Response Code: This present case demonstrates and discusses such Website: an occurrence. www.heartviews.org

Address for correspondence: Dr. Banu Sahin Yildiz, DOI: Department of Internal Medicine, Dr. Lutfi Kırdar Kartal Educational and Research Hospital, Istanbul, Turkey. 10.4103/1995-705X.144800 E‑mail: [email protected]

HEART VIEWS 86 Jul-Sep 14 Issue 3 / Vol 15 Yildiz, et al.: Carotid body tumor and chronic hypoxia autoantibodies and 24 h urinary metanephrines, vascularized connective tissue [Figures 2a, b, and 3]. vanillylmandelic acid and 131I meta‑iodobenzylguanidine The patient was discharged home in a week time scintigraphy were normal. without any complications. The patient was closely Chest X‑ray showed a cardiac enlargement and followed for the likelihood of local recurrences. diminished pulmonary vascular markings. The surface showed the right ventricular DISCUSSION hypertrophy. Transthoracic echocardiographic and cardiac catheterization evaluation revealed The association of CBTs and TOF makes interesting Tetralogy of Fallot (TOF) (A large perimembranous speculation regarding pathophysiology. The carotid ventricular septal defect, severe right ventricular body is known to be involved in the regulation of blood outflow obstruction (systolic gradient 206 mmHg), right pressure, heart rate and respiration.[1] The carotid body, ventricular hypertrophy and overriding of the ). as a chemoreceptor, responds to PO2 via response of (%) was 65%. Coronary angiography type I (glomus) cells to the stimulus and triggers an was normal. Magnetic resonance imaging showed in the carotid sinus , which is an a 16 mm × 10 mm hyperintense mass in the right carotid . The carotid sinus nerve relays the artery bifurcation [Figure 1]. information to the central nervous system. The carotid Prior to the surgery, patient received an alpha body also senses changes in the arterial pH and PCO2 blockade treatment for 2 weeks time. She underwent but to a lesser extent than PO2. The carotid body is simultaneously successful surgical resection of the very sensitive to PO2 levels in the blood; when the tumor and complete repair of the TOF (consists of PO2 is below 100 mmHg than the activity of type I cells patch closure of the ventricular septal defect and increases rapidly.[1] The type I cells in the carotid are widening of the right ventricular outflow tract; systolic derived from neuroectoderm and are thus electrically gradient decreased from 206 mmHg to 30 mmHg) excitable. after preoperative alpha blockade for 2 weeks. Macroscopy and histopathology (with H and E stain) was consistent with carotid body tumor and it is composed of nest of epitheliod cells with granular eosinophilic cytoplasm, separated by trabeculated

a

b Figure 2: (a) Localization of the carotid body tumor between and ; (b) Resection Figure 1: Magnetic resonance imaging of the carotid body tumor of the carotid body tumor

87 HEART VIEWS Jul-Sep 14 Issue 3 / Vol 15 Yildiz, et al.: Carotid body tumor and chronic hypoxia

as the pathophysiologic basis for the enlargement of the carotid body size.[4,5] Nissenblatt[4] described the development of a CBT in a 28‑year‑old woman with hypoplastic right heart syndrome and suggested that the high altitude habitation induces hyperplasia of the CBT. In TOF, the ventricular septal defect is typically large; thus, systolic pressures in the right and left ventricles are the same. Pathophysiology depends on the degree of right ventricular outflow obstruction. A mild obstruction may produce a left‑to‑right shunt through the ventricular septal defect; however, a severe obstruction, as in our case, produces a right‑to‑left shunt, resulting in low systemic arterial saturation. This hypoxia may cause the development of hyperplasia of the carotid body or a tumor. Figure 3: Histopathology of carotid body tumor CONCLUSION

A decrease in PO2, an increase in PCO2 and a decrease in arterial pH can all cause depolarization In patients with congenital cyanotic heart diseases, of the cell membrane and the effect is produced by the carotid body may enlarge due to hypoxia. This may blocking currents. This reduction in the be caused by right to left shunts or inadequate blood opens voltage‑gated flow to the lung parenchyma. channels that cause a rise in intracellular calcium concentration. This causes exocytosis of vesicles REFERENCES containing a variety of such as , , , noradrenaline 1. Gonzalez C, Almaraz L, Obeso A, Rigual R. Carotid body and .[1,3] The feedback from the carotid : From natural stimuli to sensory discharges. body is produced as an action potential and it is sent to Physiol Rev 1994;74:829‑98. the cardiorespiratory centers in the 2. Shamblin WR, ReMine WH, Sheps SG, Harrison EG Jr. Carotid body tumor (chemodectoma). Clinicopathologic analysis of ninety through the afferent branches of the glossopharyngeal cases. Am J Surg 1971;122:732‑9. nerve. These centers, in turn, regulate respiration and 3. Rodríguez‑Cuevas S, López‑Garza J, Labastida‑Almendaro S. .[2] All of these events causes increased Carotid body tumors in inhabitants of altitudes higher than 2000 firing of the chemoreceptors and subsequently lead meters above sea level. Head Neck 1998;20:374‑8. to the development of increased ventilation rate, 4. Nissenblatt MJ. Cyanotic heart disease: “Low altitude” risk for increased peripheral and possibly carotid body tumor? Johns Hopkins Med J 1978;142:18‑22. 5. Hirsch JH, Killien FC, Troupin RH. Bilateral carotid body tumors tachycardia. and cyanotic heart disease. AJR Am J Roentgenol 1980;134:1073‑5. Recent studies have pointed out to a direct correlation between increased carotid body size and an increased Source of Support: Nil, Conflict of Interest: None declared. right ventricular weight with a possible cause of hypoxia

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