e-Herz: Case study Herz 2014 DOI 10.1007/s00059-014-4079-0 Received: 24 December 2013 Revised: 4 February 2014 Accepted: 6 February 2014 © Urban & Vogel 2014
e-Herz
M.A. Rana · B. Sarwar · H. Zabher · N.I. Akkus Louisiana State University, Shreveport
Large arteriovenous malformation of the renal artery causing congestive heart failure A 45-year-old African-American female patient with uncontrolled hypertension and multiple sclerosis presented with worsening shortness of breath, dyspnea on exertion, orthopnea, and lower extremity edema over the last several months. On physical examination the patient had jugular venous distension, apical holosystolic murmur, rales in the lung bases, and grade 2+ edema in the lower extremities. Bruit was also discernable in the right upper quadrant. A transthoracic echocardiogram showed dilated right, left atrium and normal systolic function, mild diastolic dysfunction, mild mitral and tricuspid insufficiency, and an interatrial septal aneurysm with a negative jet in the bubble study due to high inferior vena cava (IVC) flow (. Fig. 1). A transesophageal echocardiogram confirmed these findings and there was no evidence of an intracardiac shunt. A re-
nal Doppler ultrasound showed normal size and velocities of the left renal artery but abnormal focal dilatation of the right renal artery with increased arterial flow velocities in both systole and diastole with spectral broadening (. Fig. 2). A computed tomography (CT) angiogram showed a large right renal artery arteriovenous malformation (AVM) with early enhancement and dilation of the intrahepatic IVC (. Fig. 3). Angiography of the right renal artery showed renal AVM draining into the IVC (. Fig. 4). In summary, this is a case of a young female patient with high-output congestive heart failure and normal left ventricular systolic function with uncontrolled hypertension secondary to a renal AVM. The symptoms improved by medical treatment but the patient declined any invasive surgical interventions for treatment of the AVM.
Fig. 1 7 Apical echocardiogram in fourchamber view depicting interatrial aneurysm and bubbles confined to the right atrium and ventricle Herz 2014
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e-Herz: Case study
Fig. 2 8 Renal Doppler ultrasound image shows normal size and velocities of left renal artery but abnormal focal dilatation of right renal artery with increased arterial flow velocities in both systole and diastole with spectral broadening
Fig. 3 8 a–d Computed tomography angiogram shows a large right renal artery arteriovenous malformation (AVM) with early enhancement and dilation of the intrahepatic inferior vena cava (IVC)
Fig. 4 8 Angiography of the right renal artery showing renal arteriovenous (AV) malformation draining into the inferior vena cava (IVC)
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Herz 2014
Renal AVMs are congenital communications between arteries and veins through a vascular nidus with a bypass of the capillary bed [1, 2]. The prevalence is reported to be less than 0.04% [3] and they are subclassified as cirsoid, angiomatous, and aneurysmal AVMs [1]. Renal arteriovenous fistulas (AVFs) are more commonly seen than renal AVMs; they occur mostly after traumatic or iatrogenic injury of the kidney and have a single direct communication between an artery and a vein without a vascular nidus [1, 4]. With the presence of any AVM or AVF there is a parenchymal steal and relative hypoperfusion distal to AVM/ AVF, which can lead to activation of the sympathetic and renin-angiotensin system. Angiotensin (AT) II activates its AT1 receptor, which induces vasoconstriction, renal sodium reabsorption, cell proliferation, cell growth, and aldosterone secretion and contributes to the development of fluid retention and hypertension [5]. Renin-mediated hypertension, hematuria, renal colic, cardiomegaly, and congestive heart failure are common manifestations of renal AVMs [6]. Examination findings can include frank bruit and a palpable mass in the setting of a renal tumor. In the large AVMs formed after a renal biopsy, the symptoms can start as early as the first weeks or months after the procedure with development of hypertension and congestive heart failure [7]. Patients with large peripheral arterial AVFs have elevated cardiac output (CO) with increased heart rate at baseline, and by compression of the AVFs the heart rate slowed down (Branham’s sign) and CO decreased [8, 9]. Exaggerated increase in cardiac output during exercise in patients with peripheral high-flow AVMs has also been reported in a recent study [10]. All these neurohormonal and hemodynamic effects of AVMs contribute to the findings seen in these patients. Symptomatic patients can benefit from closure of AVMs. The surgical methods include ligation of the renal artery with or without partial or total nephrectomy. Transcatheter coil, ethanol, gelatin sponge, Amplatzer (St. Jude Medical, Eschborn, Germany) vascular plug embolization to the AVM or covered stent placement across the AVM can
be performed as a definite procedure or to reduce the size of the fistula for further more limited surgery [7, 11].
Corresponding address M.A. Rana Louisiana State University 1333 Coates Bluff drive, Apt # 1214, 71104 Shreveport Louisiana USA [email protected]
Compliance with ethical guidelines Conflict of interest. M.A. Rana, B. Sarwar, H. Zabher, and N.I. Akkus state that there are no conflicts of interest. The accompanying manuscript does not include studies on humans or animals.
References 1. Cura M, Elmerhi F, Suri R et al (2010) Vascular malformations and arteriovenous fistulas of the kidney. Acta Radiol 51:144–149 2. Kato T, Takagi H, Ogaki K et al (2006) Giant renal aneurysm with arteriovenous fistula. Heart Vessels 21:270–272 3. Cho KJ, Stanley JC (1978) Non-neoplastic congenital and acquired renal arteriovenous malformations and fistulas. Radiology 129:333–343 4. Sakoda T, Nishimukai A, Tsujino T et al (2007) Two giant renal aneurysms and renal arteriovenous fistula associated with cardiac insufficiency and a sustained elevation of atrial natriuretic peptide and brain natriuretic peptide. Am J Med Sci 333:300–304 5. Carey RM (2013) Newly discovered components and actions of the renin-angiotensin system. Hypertension 62:818–822 6. Crotty KL, Orihuela E, Warren MM (1993) Recent advances in the diagnosis and treatment of renal arteriovenous malformations and fistulas. J Urol 150:1355–1359 7. Sasaki N, Joashi UC, Vergara M et al (2009) Postrenal biopsy AVM leading to severe hypertension and dilated cardiomyopathy. Pediatr Nephrol 24:2459–2462 8. Cohen SM, Edholm OG, Howarth S et al (1948) Cardiac output and peripheral blood flow in arteriovenous aneurysm. Clin Sci (Lond) 7:35–47 9. Branham HH (1890) Aneurismal varix of the femoral artery and vein following a gunshot wound. Int J Surg 3:250 10. Mardimae A, Han JS, Preiss D et al (2011) Exaggerated increase in cardiac output during exercise in patients with peripheral high-flow arteriovenous malformations. J Vasc Interv Radiol 22:40–46 11. Benson DA, Stockinger ZT, McSwain NE Jr (2005) Embolization of an acute renal arteriovenous fistula following a stab wound: case report and review of the literature. Am Surg 71:62–65
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e-Herz
M.A. Rana · B. Sarwar · H. Zabher · N.I. Akkus Louisiana State University, Shreveport
Large arteriovenous malformation of the renal artery causing congestive heart failure A 45-year-old African-American female patient with uncontrolled hypertension and multiple sclerosis presented with worsening shortness of breath, dyspnea on exertion, orthopnea, and lower extremity edema over the last several months. On physical examination the patient had jugular venous distension, apical holosystolic murmur, rales in the lung bases, and grade 2+ edema in the lower extremities. Bruit was also discernable in the right upper quadrant. A transthoracic echocardiogram showed dilated right, left atrium and normal systolic function, mild diastolic dysfunction, mild mitral and tricuspid insufficiency, and an interatrial septal aneurysm with a negative jet in the bubble study due to high inferior vena cava (IVC) flow (. Fig. 1). A transesophageal echocardiogram confirmed these findings and there was no evidence of an intracardiac shunt. A re-
nal Doppler ultrasound showed normal size and velocities of the left renal artery but abnormal focal dilatation of the right renal artery with increased arterial flow velocities in both systole and diastole with spectral broadening (. Fig. 2). A computed tomography (CT) angiogram showed a large right renal artery arteriovenous malformation (AVM) with early enhancement and dilation of the intrahepatic IVC (. Fig. 3). Angiography of the right renal artery showed renal AVM draining into the IVC (. Fig. 4). In summary, this is a case of a young female patient with high-output congestive heart failure and normal left ventricular systolic function with uncontrolled hypertension secondary to a renal AVM. The symptoms improved by medical treatment but the patient declined any invasive surgical interventions for treatment of the AVM.
Fig. 1 7 Apical echocardiogram in fourchamber view depicting interatrial aneurysm and bubbles confined to the right atrium and ventricle Herz 2014
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e-Herz: Case study
Fig. 2 8 Renal Doppler ultrasound image shows normal size and velocities of left renal artery but abnormal focal dilatation of right renal artery with increased arterial flow velocities in both systole and diastole with spectral broadening
Fig. 3 8 a–d Computed tomography angiogram shows a large right renal artery arteriovenous malformation (AVM) with early enhancement and dilation of the intrahepatic inferior vena cava (IVC)
Fig. 4 8 Angiography of the right renal artery showing renal arteriovenous (AV) malformation draining into the inferior vena cava (IVC)
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Herz 2014
Renal AVMs are congenital communications between arteries and veins through a vascular nidus with a bypass of the capillary bed [1, 2]. The prevalence is reported to be less than 0.04% [3] and they are subclassified as cirsoid, angiomatous, and aneurysmal AVMs [1]. Renal arteriovenous fistulas (AVFs) are more commonly seen than renal AVMs; they occur mostly after traumatic or iatrogenic injury of the kidney and have a single direct communication between an artery and a vein without a vascular nidus [1, 4]. With the presence of any AVM or AVF there is a parenchymal steal and relative hypoperfusion distal to AVM/ AVF, which can lead to activation of the sympathetic and renin-angiotensin system. Angiotensin (AT) II activates its AT1 receptor, which induces vasoconstriction, renal sodium reabsorption, cell proliferation, cell growth, and aldosterone secretion and contributes to the development of fluid retention and hypertension [5]. Renin-mediated hypertension, hematuria, renal colic, cardiomegaly, and congestive heart failure are common manifestations of renal AVMs [6]. Examination findings can include frank bruit and a palpable mass in the setting of a renal tumor. In the large AVMs formed after a renal biopsy, the symptoms can start as early as the first weeks or months after the procedure with development of hypertension and congestive heart failure [7]. Patients with large peripheral arterial AVFs have elevated cardiac output (CO) with increased heart rate at baseline, and by compression of the AVFs the heart rate slowed down (Branham’s sign) and CO decreased [8, 9]. Exaggerated increase in cardiac output during exercise in patients with peripheral high-flow AVMs has also been reported in a recent study [10]. All these neurohormonal and hemodynamic effects of AVMs contribute to the findings seen in these patients. Symptomatic patients can benefit from closure of AVMs. The surgical methods include ligation of the renal artery with or without partial or total nephrectomy. Transcatheter coil, ethanol, gelatin sponge, Amplatzer (St. Jude Medical, Eschborn, Germany) vascular plug embolization to the AVM or covered stent placement across the AVM can
be performed as a definite procedure or to reduce the size of the fistula for further more limited surgery [7, 11].
Corresponding address M.A. Rana Louisiana State University 1333 Coates Bluff drive, Apt # 1214, 71104 Shreveport Louisiana USA [email protected]
Compliance with ethical guidelines Conflict of interest. M.A. Rana, B. Sarwar, H. Zabher, and N.I. Akkus state that there are no conflicts of interest. The accompanying manuscript does not include studies on humans or animals.
References 1. Cura M, Elmerhi F, Suri R et al (2010) Vascular malformations and arteriovenous fistulas of the kidney. Acta Radiol 51:144–149 2. Kato T, Takagi H, Ogaki K et al (2006) Giant renal aneurysm with arteriovenous fistula. Heart Vessels 21:270–272 3. Cho KJ, Stanley JC (1978) Non-neoplastic congenital and acquired renal arteriovenous malformations and fistulas. Radiology 129:333–343 4. Sakoda T, Nishimukai A, Tsujino T et al (2007) Two giant renal aneurysms and renal arteriovenous fistula associated with cardiac insufficiency and a sustained elevation of atrial natriuretic peptide and brain natriuretic peptide. Am J Med Sci 333:300–304 5. Carey RM (2013) Newly discovered components and actions of the renin-angiotensin system. Hypertension 62:818–822 6. Crotty KL, Orihuela E, Warren MM (1993) Recent advances in the diagnosis and treatment of renal arteriovenous malformations and fistulas. J Urol 150:1355–1359 7. Sasaki N, Joashi UC, Vergara M et al (2009) Postrenal biopsy AVM leading to severe hypertension and dilated cardiomyopathy. Pediatr Nephrol 24:2459–2462 8. Cohen SM, Edholm OG, Howarth S et al (1948) Cardiac output and peripheral blood flow in arteriovenous aneurysm. Clin Sci (Lond) 7:35–47 9. Branham HH (1890) Aneurismal varix of the femoral artery and vein following a gunshot wound. Int J Surg 3:250 10. Mardimae A, Han JS, Preiss D et al (2011) Exaggerated increase in cardiac output during exercise in patients with peripheral high-flow arteriovenous malformations. J Vasc Interv Radiol 22:40–46 11. Benson DA, Stockinger ZT, McSwain NE Jr (2005) Embolization of an acute renal arteriovenous fistula following a stab wound: case report and review of the literature. Am Surg 71:62–65
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