229Saudi Journal of Medicine & Medical Sciences | Vol. 2 | Issue 3 | December 2014 | 229-231

Complete Heart Block with Ventricular Tachycardia in a Patient with Hemochromatosis

Kalathingathodika Sajeer, Mangalachulli P. Ranjith, Kader Muneer, Chakanalil G. SajeevDepartment of Cardiology, Government Medical College, Kozhikode, Kerala, India

Correspondence: Dr. Sajeer Kalathingathodika, Department of Cardiology, Government Medical College, Kozhikode - 673 008, Kerala, India. E-mail: drsajeerkt@gmail.com

revealed smooth, diffusely pigmented skin with absence of chest, facial, axillary and pubic hair. There was no hepatosplenomegaly and his testes and penis were small. His heart rate was 40 beats/min with a blood pressure of 120/70 mmHg. Examination of the cardiovascular system showed intermittent cannon waves, cardiomegaly, variable intensity of fi rst heart sound and normal second heart sound.

Laboratory investigations showed a hematocrit value of 50%, hemoglobin of 11 gm/dL, platelet count of 200,000/mm3, absolute neutrophil count of 7600/mm3, serum creatinine of 0.6 mg/dL, sodium of 140 meq/L, potassium of 3.4 meq/L, calcium of 9.4 mg/dL and magnesium of 2.1 meq/L. Liver function test and thyroid function tests were normal. His blood sugar was elevated. Serum iron was elevated to 312 μg/dL (N = 50-150 μg/dL) with a total iron binding capacity of 268 μg/dL (N = 250-400 μg/dL), transferrin saturation of 70.5% (N = 15-30%) and serum ferritin of 6727 ng/mL (N = 10-150 ng/mL). His electrocardiogram at admission showed complete heart block with a wide QRS escape rhythm at a rate of 36 beats/min. During hospital stay, he developed several episodes of non-sustained monomorphic ventricular tachycardia [Figure 1]. There was cardiomegaly in the

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DOI:10.4103/1658-631X.142586

A B S T R A C T

Hereditary hemochromatosis is an inherited condition of dysregulated iron absorption, and usually presents with clinical features of hepatic dysfunction. Cardiac involvement as the presenting manifestation of hereditary hemochromatosis is rare. We report a young male who presented with complete heart block and ventricular tachycardia and was subsequently diagnosed as hemochromatosis. He was managed with permanent pacemaker implantation, oral antiarrhythmic and chelation therapy.

Key words: Complete heart block, hemochromatosis, ventricular tachycardiaملخص البحث :

يمثل داء ترسب االصبغة الدموية الوراثي حالة امتصاص غير منظم للحديد. وتظهر أعراضه كخلل في وظائف الكبد. وتعتبر إصابة القلب بهذا الداء من الحاالت النادرة. يناقش الباحثون حالة مريض يعاني من حصار كامل لجهاز إيصال الكهرباء للقلب وتسارع في دقات القلب البطيني. وقد

تم عالج المريض بزرع جهاز تنظيم ضربات القلب وأدوية لتنظيم ضرباته.

INTRODUCTION

Incidence of cardiac arrhythmias is only marginally increased in asymptomatic subjects with hereditary hemochromatosis.[1] Complete atrioventricular block and ventricular tachycardia as presenting manifestations are very rare. We report a young male with complete AV block and non-sustained ventricular tachycardia who was found to have hemochromatosis.

CASE REPORT

A 30-year-old male presented with a 2-day history of recurrent syncope. He noticed diffuse pigmentation of the skin since the age of 20 years. Physical examination

CASE REPORT

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Sajeer, et al.: Hemochromatosis with complete heart block

Saudi Journal of Medicine & Medical Sciences | Vol. 2 | Issue 3 | December 2014 230

overload with secondary tissue damage in a wide range of organs. Initial symptoms often include lethargy, arthralgia, change in skin color, loss of libido and features of diabetes mellitus. Hepatomegaly, splenomegaly, arthropathy, congestive heart failure and cardiac arrhythmias are common in advanced disease. Cardiac involvement is the presenting manifestation in about 15% of the symptomatic patients.[2] The most common manifestation is congestive heart failure, which occurs in about 10% of young adults with the disease, especially those with juvenile hemochromatosis. Symptoms of congestive heart failure may develop suddenly, with rapid progression to death if untreated. The heart is diffusely enlarged; this may be misdiagnosed as idiopathic cardiomyopathy if other overt manifestations are absent.

chest skiagram and echocardiography showed mild left ventricular systolic dysfunction.

He was managed with permanent pacemaker implantation. Post-implantation, he again developed recurrent episodes of monomorphic non-sustained VT [Figure 2], for which he was started on oral amiodarone therapy. He improved with treatment and was discharged on oral iron chelation therapy and amiodarone.

DISCUSSION

Hemochromatosis is a common inherited disorder of iron metabolism, characterized by excessive iron deposition in parenchymal cells that can lead to total-body iron

Figure 1: Electrocardiogram showing complete AV block and non-sustained VT

Figure 2: Electrocardiogram showing ventricular paced beats with ventricular premature contractions in couplets

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Sajeer, et al.: Hemochromatosis with complete heart block

231Saudi Journal of Medicine & Medical Sciences | Vol. 2 | Issue 3 | December 2014

Cardiac arrhythmias include premature supraventricular beats, paroxysmal tachyarrhythmia, atrial fl utter, atrial fi brillation and varying degrees of atrioventricular block.[3-5] Various A-V blocks and atrial arrhythmias are reported in patients with hemochromatosis, but complete heart block with ventricular tachycardia is a rare occurence.[6,7] A study among the urban male population of Sweden showed a prevalence of iron overload in the pacemaker-treated population, where high-grade AV block was less than 2%.[8] Animal studies have shown that chronic iron overload decreases CaV1.3-dependent L-type Ca2+ currents, leading to bradycardia, altered electrical conduction and atrial fi brillation.[9]

CONCLUSION

Our patient had the classical clinical and laboratory manifestations of hemochromatosis, and he completely responded to permenent pacemaker implantation followed by oral amiodarone therapy. Asymptomatic male with hemochromatosis presenting with complete heart block and ventricular tachycardia are the highlights in this case.

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8. Rosenqvist M, Hultcrantz R. Prevalence of a haemochromatosis among men with clinically signifi cant bradyarrhythmias. Eur Heart J 1989;10:473-8.

9. Rose RA, Sellan M, Simpson JA, Izaddoustdar F, Cifelli C, Panama BK, et al. Iron overload decreases CaV1.3-dependent L-type Ca2+ currents leading to bradycardia, altered electrical conduction, and atrial fi brillation. Circ Arrhythm Electrophysiol 2011;4:733-42.

How to cite this article: Kalathingathodika S, Mangalachulli RP, Kader M, Chakanalil SG. Complete heart block with ventricular tachycardia in a patient with hemochromatosis. Saudi J Med Med Sci 2014;2:229-31.

Source of Support: Nil, Confl ict of Interest: None declared.

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