case report Wien Klin Wochenschr DOI 10.1007/s00508-013-0476-2

A 56-year-old man with co-prevalence of Leriche syndrome and dilated cardiomyopathy: case report and review Karsten Keller · Johannes Beule · Jörn Oliver Balzer · Meike Coldewey · Thomas Munzel · Wolfgang Dippold · Philipp Wild

Received: 26 October 2012 / Accepted: 13 November 2013 © Springer-Verlag Wien 2013

Summary  We report about a 56-year-old man with dyspnoea and leg pain diagnosed with Leriche syndrome and chronic heart failure caused by dilated cardiomyopathy (DCM) with acute cardiac decompensation. Optimising of chronic heart failure therapy with diuretic and antihypertensive drugs leaded to recompensation. A defibrillator was implanted, and afterwards surgical therapy of Leriche syndrome was planned. Leriche syndrome is an uncommon variant of atherosclerotic occlusive disease characterised by total occlusion in abdominal aorta and/or both iliac arteries. If aortic stenosis develops slowly, collateral vascular circulation The patient of this case report was hospitalised in the Department of Internal Medicine, St. Vincenz and Elisabeth Hospital Mainz (KKM) (Head: Prof. Dr. med. W. Dippold), and the Department of Medicine II, University Medical Center Mainz (Johannes Gutenberg-University Mainz) (Head: Univ.-Prof. Dr. med. T. Munzel). Dr. med. K. Keller, MD () · M. Coldewey, MD · Univ.-Prof. Dr. med. T. Munzel, MD · Univ.-Prof. Dr. med. P. Wild, MD, MSc Department of Medicine II, University Medical Center Mainz, Johannes Gutenberg-University Mainz, Langenbeckstr. 1, 55131 Mainz, Germany e-mail: [email protected] Dr. med. K. Keller, MD · M. Coldewey, MD · Univ.-Prof. Dr. med. T. Munzel, MD · Univ.-Prof. Dr. med. P. Wild, MD, MSc Centrum for Thrombosis and Haemostasis, University Medical Center Mainz, Johannes Gutenberg-University Mainz, Mainz, Germany Dr. med. J. Beule, MD · Prof. Dr. med. W. Dippold, MD Department of Internal Medicine, St. Vincenz and Elisabeth Hospital Mainz, Katholisches Klinikum Mainz (KKM), Mainz, Germany Priv.-Doz. Dr. med. J. O. Balzer, MD Department of Radiology and Nuclear Medicine, Katholisches Klinikum Mainz (KKM), Mainz, Germany

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can be found frequently. Typical symptoms are claudication, symptoms related to an arterial insufficiency of the lower extremities, erectile dysfunction and weight loss. Risk factors of Leriche syndrome are diabetes mellitus, hypertension, hyperlipaemia and smoking. Further it is often associated with chronic renal failure and coronary artery disease. Diagnosis is normally made by computed tomography (CT) or magnetic resonance imaging (MRI). Standard therapy is surgical revascularisation. DCM is a common cause of a congestive heart failure, which could be induced by coronary artery disease, hypertension, toxic, metabolic, inflammatory and infectious agents, and inherited gene defects. Keywords  Leriche syndrome · Atherosclerotic occlusive disease · Claudication · Dilated cardiomyopathy

56-jähriger Mann mit gleichzeitigem Vorkommen eines Leriche Syndroms und einer dilatativen Kardiomyopathie Zusammenfassung  Wir berichten über den Fall eines 56-jährigen Patienten, der sich wegen Dyspnoe und Beinschmerzen in der Notaufnahme vorstellte. Verursacht wurden die Beschwerden durch ein Leriche Syndrom und eine dekompensierte Herzinsuffizienz auf dem Boden einer dilatativen Kardiomyopathie. Durch eine Optimierung der Herzinsuffizienztherapie mit unter anderem dem Einsatz von diuretisch und antihypertensiv wirksamen Medikamenten konnte eine Rekompensation erreicht werden. Ein Leriche Syndrom stellt eine seltene Variante der atherosklerotischen Verschlusskrankheit dar, charakterisiert durch einen kompletten Verschluss der abdominellen Aorta und/oder beider Aa. iliacae. Wenn sich die Aortenstenose über einen langen Zeitraum entwickelt, kann meist eine Kollateralenbildung beobachtet wer-

A 56-year-old man with co-prevalence of Leriche syndrome and dilated cardiomyopathy: case report and review  

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den. Typische Symptome eines Leriche Syndroms sind Claudicatio-Schmerzen und andere Symptome verursacht durch die Unterversorgung der unteren Extremitäten, erektile Dysfunktion und Gewichtsverlust. Risikofaktoren eines Leriche Syndroms sind Diabetes mellitus, Arterielle Hypertonie, Hyperlipidämie und Rauchen. Weiterhin sind häufig Assoziationen des Syndroms zu einer chronischen Niereninsuffizienz und einer koronaren Herzkrankheit zu bemerken. Die Diagnose wird in den meisten Fällen mittels Computer-Tomographie oder Magnet-Resonanz-Tomographie gestellt. Standardtherapie ist die gefäßchirurgische Revaskularisierung. Eine dilatative Kardiomyopathie ist ein häufiger Grund einer chronischen Herzinsuffizienz, welche sowohl durch eine koronare Herzkrankheit und eine Arterielle Hypertonie als auch durch toxische, metabolische, entzündliche und infektiöse Agens und durch vererbte Gendefekte ausgelöst werden kann. Schlüsselwörter  Leriche Syndrom  · Atherosklerotische Verschlusskrankheit  · Claudicatio  · Dilatative Kardiomyopathie

Case report Clinical presentation on admission A 56-year old man presented at the emergency department with progressive dyspnoea at rest, oedema, pain of the legs and abdominal discomfort. Dyspnoea had persisted since 2 years on physical activities (NYHA II), and showed a progression to NYHA IV in the last 5 days. The patient suffered from an impaired walking capacity with walking distance limited to 100 m due to intermittent claudication. Moreover, the patient reported postprandial abdominal discomfort with pain and meteorism and weakness of the left arm in physical activities. Syncope or collapse was not reported. Co-morbidities comprised a not well-controlled diabetes mellitus type 2, continued smoking with 60–70 pack years (PY), arterial hypertension, chronic renal failure, hyperlipidemia and an alcohol addiction with abstinence for 10 years. Six months ago, the patient’s general practitioner had diagnosed him with chronic heart failure, and started an appropriate drug therapy with an angiotensin-converting enzyme inhibitor (Ramipril).

Examinations and findings Physical examination showed a reduced general condition. Body mass index was elevated to 34 kg/m2. Although patient had dyspnoea at rest, there were no visible signs of cyanosis. Auscultation of the lungs revealed expiratory rales throughout the basal lung fields. Oxygen saturation was measured peripherally with 95 % without administration of oxygen. Heart rate was 91 beats/min and regular. Heart auscultation revealed a stenosal cardiac murmur

of the aortic valve and regurgitant cardiac murmurs of mitral and tricuspid valve. Both legs showed oedema and trophic dermal alterations as signs of chronic oedema without ulcer or gangrene. Abdominal examination was without pathological findings. Pulses of carotid arteries and right arm were palpable, whereas pulses of left arm and lower extremities were not palpable. All extremities were warm and with regular skin complexion. Blood pressure differed between left and right arm (right 120/80 mmHg, left 75/50 mmHg). The patient had no impairment or loss of sensibility, coordination and motor functions. Laboratory analyses revealed an increased creatinine (1.5 mg/dl; normal: 0.7–1.3 mg/dl). All other parameters including troponin and creatine kinase were in reference range. Electrocardiogram (ECG) showed sinus rhythm (91/ min) with anterolateral negative T in I, aVL, v5, v6 and positive Sokolow–Lyon index. In echocardiography, all heart chambers were dilated, left ventricle was hypertrophied (interventricular septum measured 14  mm) and left ventricular ejection fraction (LVEF) was reduced (20 %). Valves were altered and valve insufficiency with a middle degree of severity of aortic, mitral and tricuspidal valves was detected. Systolic pulmonary artery pressure (sPAP) was elevated (45 mmHg). Measurement of ankle–brachial index suggested a peripheral arterial disease of middle-degree severity (right: 0.55; left: 0.54). X-ray examination of the chest presented an enlarged heart and a pulmonary venous congestion in the lungs.

Differential diagnosis on admission 1. Chronic heart failure with acute cardial decompensation 2. Dilated cardiomyopathy (DCM) 3. Cor hypertensivum 4. Suspect of peripheral arterial disease differential diagnosis aortic stenosis 5. Suspect of subclavian steal syndrome

In-patient diagnostics and treatment Due to acute cardial decompensation, the patient was treated with diuretic drugs, and therapy of chronic heart failure and hypertension was optimised. Further imaging diagnostics were initiated to clarify the suspect of peripheral arterial disease and/or aortic stenosis and suspect of subclavian steal syndrome. MR-angiography of aortic arch and supra-aortic vessels demonstrated a severe stensosis of the left subclavian artery, while other vessels revealed no pathology. Abdominal aorta showed an occlusion of infrarenal aorta and both common iliac arteries. Both internal iliac arteries as well as right external iliac artery were reperfused through collateral vessels (Figs. 1 and 2). Vessel stenosis

2   A 56-year-old man with co-prevalence of Leriche syndrome and dilated cardiomyopathy: case report and review

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Fig. 1  Leriche syndrome with occlusion of the infrarenal aorta. The occlusion was placed directly beyond renal branches (magnetic resonance imaging picture)

of A. mesenterica superior, A. mesenterica inferior or extremities arteries were not recognised. Duplex ultrasound did not show a significant stenosis of Aa. carotis interna, but showed a distinct sclerosis of the Aa. carotis externa. Aa. vertebralis flow was detectable in both arteries. In rest, a flow reversal in the left A. vertebralis vessel was not recognised. Coronary angiography was done via right radial artery. Significant coronary artery disease was excluded. Ventriculography displayed a global hypokinesia with distinctly decreased LVEF of 15 %.

Confirmed diagnosis 1. Acute cardiac decompensation of a DCM with congestive heart failure –– LVEF 15 %, sPAP 45 mmHg –– Middle-degree insufficiency of aortic, mitral and tricuspidal valve 2. Cor hypertensivum with left ventricular muscle hypertrophy and long-standing arterial hypertension 3. Atherosclerotic occlusive disease with Leriche syndrome –– O  cclusion of the infrarenal abdominal aorta and both Aa. iliacae communis

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Fig. 2  Magnetic resonance imaging showed an occlusion of the infrarenal aorta and both common iliac arteries. Both internal iliac arteries as well as the right external iliac artery were reperfused though collateral vessels

–– Subtotal occlusion of the left A. subclavia with symptomatic of subclavian steal syndrome 4. Cardiovascular risk factors and co-morbidities: continued smoking (60–70 PY), arterial hypertension, not well-adjusted diabetes mellitus type 2, dyslipidemia and chronic renal failure

The followed course Acute cardial decompensation was regressive under intravenous diuretic and optimised oral drug therapy of heart failure. Recompensation with reduction of body weight (6.4 kg), dyspnoea and oedema was achieved. Moreover, antidiabetic and antihypertensive therapies were newly adjusted. Treatment with acetylsalicylic acid and statine was started. The patient was encouraged to quit smoking. Ischemic genesis of the DCM was excluded via coronary angiography. It was assumed that DCM was induced by the prevalent arterial hypertension and diabetes mellitus combined with alcohol abuse in the past. Implantable cardio-defibrillator was implanted for prognostics reasons, because of poor left ventricular function. Afterwards, surgery of Leriche syndrome was planned. Axillo-femoral bypass or percutaneous transluminal angioplasty was favoured.

A 56-year-old man with co-prevalence of Leriche syndrome and dilated cardiomyopathy: case report and review  

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case report Discussion Leriche syndrome was first described by Quain in 1847 and later on by Leriche and Morel in 1940 [1–3]. It is an uncommon vascular pathology characterised by total occlusion of abdominal aorta and/or both iliac arteries [1–7]. Incidence and aetiology are unknown [1]. Leriche syndrome is a variant of atherosclerotic occlusive disease [1, 4, 7]. It was also suggested that Leriche syndrome is caused by developmental defect of aortic growth, resulting in hypoplastic infrarenal aorta [1]. Other described aetiologies are exposure to radiation, congenital rubella infection, luetic aortitis, Ormond’s disease, thrombangiitis obliterans Winiwarter–Buerger, inflammation and granulomatous vasculitis (Takayasu arteriitis) [1, 7]. Prevalence of peripheral arterial disease was noted between 3 and 10 % [8]. In patients over 70  life-years, prevalence increases to 20 % [8]. Inter-renal segment of aorta, the most common location of Leriche syndrome, is with 52 % [8]. In 25 % it occurs at infra-renal aorta, in 11 % occurs at suprarenal aorta and 12 % of patients reveal diffuse aortic involvement [1]. Slow development of aortic stenosis could lead to collateral vascular circulation [7]. Typical symptoms are intermittent claudication and symptoms related to an arterial insufficiency of the lower extremities (like muscle atrophy, myalgias, paleness, pain, neurologic symptoms, oedema, trophic troubles, fatigability of the lower limbs and gangrene), an aggravation of hypertension or newly recognised hypertension, erectile dysfunction and weight loss [1–3, 5, 6, 9–12]. Symptoms occur often in the 4th–5th decade of life [1, 9]. Typical risk factors of Leriche syndrome are diabetes mellitus, hypertension, hyperlipaemia and smoking [1,  5, 8, 11, 13, 14]. Furthermore, it is often associated with chronic renal failure and coronary artery disease [1, 8, 11–13]. Patients with Leriche syndrome and diabetes mellitus have more likely an advanced coronary disease than those without diabetes mellitus [12]. Physical examination typically shows differences between blood pressure of arms and legs [1]. Femoral and calf arteries are decreased or nonpalpable, and ankle–brachial indexes are lowered on both sides [1, 5, 6, 10]. Diagnosis is made by CT or MRI [1, 6–8, 15]. MRI is favourable because of better soft tissue information and discrimination of vessel walls. Additionally, MRI and MR angiography are performed without any x-ray exposure or iodine contrast material. Volume of administered contrast material is lower for MR examination than for CT examination (15–20 mL versus 80–100 mL) [7]. Standard therapy of Leriche syndrome is surgical revascularisation [1, 4, 5]. Surgical standard management options are bypass grafts and endarterectomy [1, 4, 8, 10]. Aortofemoral bypass is the most common grafting technique [1, 5, 10]. For patients with a high surgical risk techniques of axillo-femoral bypass and percutaneous transluminal angioplasty are used [10]. Further standard therapy of atherosclerotic occlusive disease with acetylsalicylic acid, statine and modification of cardiovascular risk factors are recommended [8, 16].

Atherosclerotic occlusive disease is associated with a high cardiovascular lethality [8, 15]. Up to 25 % of the patients with peripheral arterial disease die during 5  years after making the diagnosis [8, 15]. Myocardial infarction and stroke are the typical causes of increased lethality [8]. Our patient showed most typical symptoms of a slowly developed Leriche syndrome with an existing collateral vascular circulation [7]. Typical risk factors were existent [1, 5, 8]. Moreover, we diagnosed acute cardial decompensation of chronic heart disease caused by a DCM with typical symptoms [17]. DCM is a myocardial disorder that concludes ventricular dilatation and systolic contractile dysfunction [17–24]. In adults, incidence of 5.5–7.5 cases per 100,000 per year and a prevalence of 36–37 cases per 100,000 individuals has been reported [17, 22, 23]. DCM is a common cause of a congestive heart failure [18, 23]. More than 10,000 deaths are caused by DCM in the US annually [22]. Coronary artery disease, hypertension as well as DCM related to toxic, metabolic, inflammatory and infectious agents and inherited gene defects are most frequent causes [12, 17–19, 22–31]. Approximately 25–35 % of the patients with DCM have a genetic form [17, 19, 21, 23, 32]. DCM could lead to supraventricular and ventricular arrhythmias, thromboembolism and sudden or heart-failurerelated death [18, 28, 33]. In our patient’s anamnesis, no evidence for an inherited familial DCM was found. Therefore, there was no evidence for past myocarditis as another important reason for DCM [34], and endomyocardial biopsy and genetic testing were not performed [35]. As Leriche syndrome is an atherosclerotic occlusive disease in combination with diabetes mellitus and other cardiovascular risk factors, we presumed that the primary genesis of DCM would be in coronary heart disease. In contrast to our expectation, a significant coronary heart disease was excluded in the coronary angiography. By exclusion of coronary heart disease and accounting anamnesis, we determined that long-standing arterial hypertension with additional effect of increased alcohol consumption in the past and diabetes mellitus were responsible for development of DCM. Arterial hypertension and diabetes mellitus are wellknown risk factors for cardiomyopathy [14, 17, 36–42]. Arterial hypertension and coronary heart disease are responsible for three-fourths of all cases of chronic heart failure [41]. Moreover, chronic alcohol consumption is a common cause of DCM [26, 28, 31]. Exact pathogenesis is incompletely understood [20, 26]. Identified pathophysiologic mechanisms are histologic and cellular changes like: myocyte loss; intracellular organelle dysfunction; changes of contractile proteins and calcium homeostasis; and activation of sympathetic nervous system, renin– angiotensin–aldosterone system (RAAS), cytokines and natriuretic peptide system [20, 26, 28]. DCM therapy follows guidelines of chronic heart failure [17, 28–30]. Changing life habits with strict ces-

4   A 56-year-old man with co-prevalence of Leriche syndrome and dilated cardiomyopathy: case report and review

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sation of alcohol consumption and smoking, diet with reduction of salt and fluid resuscitation, and adapted physical activity are the basis of therapy. Diabetes and hypertension should be well adjusted [17, 36, 37, 41]. Prophylactic defibrillator implantation in cases of highly restricted LVEF ( 

A 56-year-old man with co-prevalence of Leriche syndrome and dilated cardiomyopathy: case report and review.

We report about a 56-year-old man with dyspnoea and leg pain diagnosed with Leriche syndrome and chronic heart failure caused by dilated cardiomyopath...
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