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We present the case of a 63-year-old woman presenting with a huge pelvic and retroperitoneal high flow arteriovenous malformation (AVM) causing high-output heart failure, who was treated with combined therapies, including transarterial embolization with n-butyl cyanoacrylate–iodized oil mixture (NBCA–lip) and coils for the right ovarian, both internal iliac, 3rd and 4th lumber arteries, venous sclerotherapy using coils and ethanolamine oleate (EO) for the right ovarian and both internal iliac veins with balloon-occluded retrograde transvenous obliteration technique, and direct percutaneous sclerotherapy using the NBCA–lip and EO for the large nidus of AVM under outflow control using occlusion balloon catheters.

Vein of Galen arterio-venous malformation is a rare congenital anomaly, which presents in the neonatal period. High output heart failure in the absence of an underlying structural heart defect points towards the condition and is an important contributing factor for morbidity and mortality. We describe a case of a term newborn who presented with respiratory distress and cardiomegaly. The characteristic echocardiographic findings of high output cardiac failure were found and eventually led to the diagnosis of an underlying vein of Galen arterio-venous malformation. The relevant literature is discussed.

Patients with end-stage renal disease (ESRD) with arteriovenous dialysis access (AVDA) can develop symptoms of heart failure and pulmonary hypertension (PH). We report on 5 patients with ESRD and AVDA who presented with shortness of breath, heart failure, and PH. All patients had partial or complete closure of AVDA and were reevaluated after AVDA revision. All 5 subjects had clinical and echocardiographic evidence of heart failure, hypertensive heart disease, left ventricular diastolic dysfunction, and PH at baseline. After complete closure ([Formula: see text]) or partial banding ([Formula: see text]) of AVDA, mean New York Heart Association class improved from [Formula: see text] to [Formula: see text] ([Formula: see text]). Mean 6-minute walk distance improved from [Formula: see text] to [Formula: see text] m ([Formula: see text]). Serial echocardiography revealed a decrease in the right ventricle∶left ventricle ratio from [Formula: see text] to [Formula: see text] ([Formula: see text]) and improved diastolic dysfunction parameters. On right heart catheterization before definitive AVDA revision, acute manual fistula or graft occlusion led to an average decrease in cardiac output of 1.1 L/min with no other changes in hemodynamics: [Formula: see text] to [Formula: see text] L/min ([Formula: see text]). However, the average decrease in cardiac output after definitive revision of the AVDA (mean, 90 days) was 4.0 L/min with marked improvements in biventricular filling pressures and pulmonary artery pressure. In patients with ESRD and AVDA presenting with heart failure and PH, revision or closure of AVDA can markedly improve dyspnea as well as the clinical, echocardiographic, and hemodynamic manifestations of heart failure and PH.

La Presse Medicale - In Press.Proof corrected by the author Available online since samedi 20 decembre 2014

A 64-year-old woman who has a history of congestive heart failure and atrial fibrillation was admitted to our hospital with the exacerbation of exertional dyspnea and urinary retention due to severe gross hematuria. Contrast-enhanced computed tomography showed a tumor involving the inferior and middle poles of the right kidney with no nodal involvement, or distant metastases, but that was accompanied by markedly proliferated blood vessels around the inferior vena cava and right renal vein, seemingly a result of an arteriovenous fistula. After embolization of the right renal artery, right radical nephrectomy was performed via a thoracoabdominal incision. The histological diagnosis of the tumor was clear cell renal cell carcinoma, G2 > G3, Fuhrman nuclear grade3, pT2a. Although the presence of an arteriovenous fistula was not confirmed histologically, the severely condensed proliferation of the blood vessels in the renal hilum is consistent with the diagnosis of an arteriovenous fistula accompanying renal cell carcinoma. Immediately after the operation, her symptoms of congestive heart failure, including dyspnea, subsided and her serum BNP levels and CTR value returned to normal levels. Two years after the operation, she shows no signs of recurrence or metastasis. To the best of our knowledge, there have been 25 cases of arteriovenous fistulas accompanied by renal cell carcinoma but only a few in which the symptoms were those of severe congestive heart failure. Clinicians should be aware that renal cell carcinoima could be a cause of heart failure.

Pulmonary hypertension (PH) associated with hereditary hemorrhagic telangiectasia (HHT) occurs most commonly as a consequence of high-output heart failure related to excessive shunting through visceral vascular malformations. A smaller number of HHT patients develop a form of heritable pulmonary arterial hypertension, characterized by an elevated pulmonary vascular resistance with normal left heart filling pressures. In this review, we will discuss the clinical manifestations of HHT in general, the pathophysiology behind the different forms of PH associated with HHT, and the clinical care of the HHT patient with PH.

A 36-year-old male presented with progressive exertional dyspnea over months. Physical examination showed jugular venous distension, lung crecipitations, femoral bruit and pitting pedal edema. Echocardiogram showed a dilated right ventricle with severe pulmonary hypertension and a non collapsing inferior vena cava (IVC). On right heart catheterization, IVC oxygen saturation was noted at 92% suggesting arterial mixing; a computed tomography of the abdomen showed a fistula between the right common iliac artery to the right common iliac vein at L4 level and a massive IVC; this was linked to trauma from a disectomy done 16 years ago at L4–L5 level. Endovascular sealing with a 16 × 60 mm bifurcated stent graft (S & G Biotech, Seoul, Korea) was performed which led to complete resolution of the patient’s dyspnea. Iatrogenic vascular injury during lumbar disc surgery, although rare, can lead to high output cardiac failure developing over months to years., Graphical Abstract

Renal arteriovenous fistulas (AVFs) are an uncommon complication of nephrectomy. In this report, we present the case of a 66-year-old female presenting with progressive dyspnea on exertion and exercise intolerance. She was diagnosed and treated for adult onset reactive airway disease. The patient underwent nephrectomy at age 18 secondary to recurrent pyelonephritis from vesicoureteral reflux. She underwent a surveillance computed tomography (CT) scan to evaluate a small ascending aneurysm that was initially detected on cardiac echocardiogram. A large left renal AVF was detected incidentally on the CT scan. The fistula was successfully treated by ligation of the renal artery with resolution of pulmonary symptoms.

Hereditary hemorrhagic telangiectasia (HHT), also known as Osler-Weber-Rendu syndrome, is a rare genetic blood disorder that leads to abnormal bleeding due to absent capillaries and multiple abnormal blood vessels known as arteriovenous malformations. A feature of HHT is high-output heart failure due to multiple arteriovenous malformations. High-output heart failure can lead to recurrent epistaxis Kiesselbach area syndrome (REKAS), further exacerbating heart failure through increased blood loss and resultant anemia. We report a patient with HHT who presented with high-output heart failure contributing to REKAS. In patients with REKAS, we propose if anemia is present, REKAS can be avoided by correcting the anemia by increasing the hemoglobin level to greater than 9 to 10 g/dL. This decreases hyperdynamic circulation and reduces pressure in the blood vessels of the nose.

The liver and the heart are closely related organs connected by large vessels. Thus, the heart may be affected by liver diseases, leading to cardiac overload, pulmonary hypertension or high-output heart failure. Severe or chronic liver diseases may result in several heart or pulmonary complications independent of any other existing cardiac or lung diseases in the patient. The major causes are cirrhosis, non-cirrhotic portal hypertension, congenital portosystemic shunts, hereditary haemorrhagic telangiectasia and congenital liver hemangioma. These liver diseases lead to various degrees of cirrhotic cardiomyopathy, vasoactive imbalance or high-flow cardiopathy. High blood flow, shunts and cardiac dilatation predominate on imaging. In the presence of a vasoactive imbalance, the hepatopulmonary syndrome and portopulmonary hypertension must be differentiated. The aim of this review was to describe the diagnostic and imaging features of heart and lung complications from liver diseases.

This chapter discusses the case of a patient who had been on dialysis for 4 years. Over the past 6 months, he has developed progressively more severe congestive heart failure manifesting as dyspnea on exertion and orthopnea. The patient’s cardiac problems have been managed by a cardiologist who felt that he was receiving maximum medical management. The patient was referred to the dialysis access center because his nephrologist and cardiologist felt that he had a high blood flow fistula which was contributing to his cardiac failure.

Background & Aims Though hepatic involvement is common in patients with hereditary haemorrhagic telangiectasia (HHT), symptomatic liver disease is rare but potentially fatal without liver transplantation. Factors associated with clinically significant liver disease in patients with HHT are unknown. Methods In this prospective cohort study, we included consecutive patients from 2001 to 2011 with definite HHT, who underwent systematic protocol screening including contrast-enhanced hepatic CT and/or abdominal ultrasound. Using a multivariable logistic regression model, we developed a simple clinical scoring index to identify the presence of symptomatic liver disease (cardiac failure, portal hypertension, or biliary disease) or ‘at-risk' liver disease (asymptomatic patients, with hepatic bruit, abnormal liver biochemistry, or elevated cardiac index). Results Of 316 patients with definite HHT, 171 patients (54.1%; age 53.4±15.2y, 101 females) had hepatic involvement on imaging. Twenty-nine patients had symptomatic liver disease (22 patients with high-output heart failure); 45 patients were ‘at-risk' for liver disease. Using multivariable logistic regression analysis, we derived a score using age, gender, hemoglobin and alkaline phosphatase at presentation which could accurately distinguish patients with clinically significant liver involvement from patients with no or incidental liver lesions (c-statistic=0.80). A score 6 indicated high risk (>80%) of harboring clinically significant liver disease in HHT. Conclusions A simple scoring system can distinguish patients at low, moderate, and high risk of harboring clinically significant liver disease. With validation, this score may be used to identify patients for individualized screening and enrollment in clinical trials.

Congestive heart failure (CHF) is an important source of morbidity and mortality in end-stage renal disease patients. Although CHF is commonly associated with low cardiac output (CO), it may also occur in high CO states. Multiple conditions are associated with increased CO including congenital or acquired arteriovenous fistulae or arteriovenous grafts. Increased CO resulting from permanent AV access in dialysis patients has been shown to induce structural and functional cardiac changes, including the development of eccentric left ventricle hypertrophy. Often, the diagnosis of high output heart failure requires invasive right heart monitoring in the acute care setting such as a medical or cardiac intensive care unit. The diagnosis of an arteriovenous access causing high output heart failure is usually confirmed after the access is ligated surgically. We present for the first time, a case for real-time hemodynamic assessment of high output heart failure due to AV access by interventional nephrology in the cardiac catheterization suite.

Background: Sickle Cell Disease (SCD) is characterized by chronic anemia and recurrent ischemia-reperfusion episodes that contribute to high output heart failure. The effects of SCD on the heart are significantly underrecognized. Methods: SCD patients who underwent echocardiography between March 2016 and March 2018 were retrospectively analyzed. Patients with reduced Left Ventricular Ejection Fraction (LVEF) and valvular heart disease were excluded. Cardiac chamber size, systolic and diastolic function parameters, and LV and RV strain were compared between hemoglobin SS (most severe form of SCD) and SC (less severe form) subtypes and against healthy controls. Wilcoxon signed rank test was used for statistical analysis. Results: SS patients (n = 48, mean age 31.9) had lower mean hemoglobin 8.9 g/dl vs 11.3 g/dl (p < 0.001) and hematocrit 25.8% vs 31.4% (p = 0.008) and higher LDH 437 IU/L vs 258 IU/L (p < 0.001) compared to SC patients (n = 11, mean age 34.4). Both SS and SC patients had worse diastolic function compared to healthy controls: higher E velocity 98.9 cm/s (SS), 86.4 cm/s (SC), 76.4 cm/s (control) (SS vs control, p < 0.01; SC vs control, p < 0.05) and higher E/A ratio 1.76 (SS), 1.59 (SC), 1.15 (control) (SS vs control, p < 0.001; SC vs control, p < 0.01). SS patients had larger indexed left atrial volume compared to SC patients (39.3 ml/m2 vs 28.4 ml/m2, p = 0.007). There was no significant difference in LVEF, left ventricular global longitudinal strain, or right ventricular strain between SS and SC subtypes compared to healthy controls. Furthermore, SS patients with a serum LDH > 500 IU/L had higher E/e' ratio (11.3 vs 7.2, p=0.001) and larger indexed Left Ventricular End Diastolic Volume (LVEDVi) (80.8 ml/m2 vs 53.4 ml/m2, p=0.002) compared to SS patients with LDH < 500 IU/L. Conclusion: SCD genotype adversely determines the degree of cardiac dysfunction in patients with SCD. LVEDVi, left atrial size, E velocity, E/A ratio, and E/e' ratio may serve as useful echocardiographic parameters to follow in this patient population. Serum LDH has been associated with poor clinical outcomes in patients with SCD, and as demonstrated by our study, it also portends worsening cardiac function in this population at high risk for heart failure. Figure Disclosures No relevant conflicts of interest to declare.

In this paper, we present the simulation of 5 different heart failures with the help of the Cardiovascular Simulation Toolbox (CVST) proposed by O. Barnea et al. at Tel-Aviv University. This is a modified version of the CVST, proposed by G.Ortiz, here, we show that the pathological failures can be covered by this tool. We varied the value of the tool blocks, included the results of the hemodynamic parameters and the P-V loop curves for each disease and compared them to the medical data to prove the effectiveness of the simulation. Based on these changes, we achieved an effective simulation of the following heart failures in the CVST: Diastolic Heart Failure (DHF), Systolic Heart Failure (SHF), Right Ventricle Heart Failure (RVHF), Low Output Heart Failure (LOHF) and High Output Heart Failure (HOHF).

High-output heart failure from arteriovenous fistula (AVF) is an often overlooked cause of pulmonary hypertension. A 63 year-old male with hepatitis C, cirrhosis, and chronic atrial fibrillation presents with worsening exertional dyspnea since a nail gun injury 7 years ago. Vitals are normal. He

High output heart failure is characterized by elevated cardiac index (CI) and decreased systemic vascular resistance (SVR). Obesity, liver disease and arteriovenous (AV) shunting are common etiologies. A 58-year-old male with history of end-stage renal disease on dialysis, alcoholic cirrhosis

A 73-year-old woman was admitted to hospital because of progressive dyspnea on exertion. Computed tomography revealed a large hepatic tumor, which was proved to be a hepatic epithelioid hemangioendothelioma (EHE). Echocardiography demonstrated high cardiac output, for which the tumor was considered to be the leading cause. A transcatheter arterial chemoembolization (TACE) was performed sequentially at 1-month intervals to reduce the size of the hepatic tumor, and this temporarily improved the patient's cardiac condition and quality of life. In this case, we successfully used TACE in the treatment of hepatic EHE with high-output heart failure. TACE is a reasonable choice of treatment both for managing malignant hepatic tumors and resolving low systemic vascular resistance by embolization of the abnormal neoangiogenic vessels. Nevertheless, clinicians should be aware of the potential adverse effect of hepatic decompensation induced by TACE, especially when the tumor involvement is widespread and poorly preserved hepatic function is encountered.

Objective Chronic intermittent renal replacement therapy(RRT) is an alternate method of decongestion for patients presenting with diuretic-resistant, end-stage heart failure(HF) and cardiorenal syndrome. The optimal method of vascular access has not been confirmed. This study investigated the 6-month outcomes of patients with end-stage HF after the creation of arteriovenous communications (AVC) compared with other means of RRT. Methods We treated 40 patients with chronic, intermittent, ambulatory RRT, of whom 15 (37.5%; Group A) underwent creation of AVC, and 25 (62.5%; Group B) received intraperitoneal (n = 6) or internal jugular catheters (n = 19) with the goal of achieving body weight stabilization and relief from congestion. Results The characteristics of the two groups were similar. According to Cox regression analysis, the 6-month rate of death or re-hospitalization for HF was significantly higher in Group A (73%) than in Group B (44%); hazard ratio (HR): 2.58; 95% confidence interval (CI) 1.2-6.2; P = 0.02. Over a 6-month follow-up, the cumulative survival was significantly shorter ( P = 0.03) in Group A (13.8 ± 10 weeks) than in Group B (20.7 ± 7 weeks). In the 15 patients who received AVC, the only independent predictor of adverse outcome at 6 months was serum total bilirubin concentration (HR 2.5; 95% CI 1.1-5.7, p = 0.02), whereas in the 25 patients who underwent other means of RRT, pulmonary vascular resistance (PVR) was identified as a risk factor for hospitalization or death at 1-year follow-up (HR 1.26; 95% CI 1.1-1.57, p = 0.04). Conclusion In patients with end-stage HF, the creation of AVC for intermittent RRT was followed by a significant increase in morbidity and mortality in comparison to the safe and effective placement of permanent central venous catheters. Patients with elevated PVR seem to comprise a group at high risk for adverse outcomes after central catheter insertion.

The syndrome of heart failure (HF) is characterized by exercise intolerance, limited by dyspnea and fatigue, and associated with neurohormonal activation and fluid retention. The primary stimulus that signals the kidney to retain salt and water is debated. In the late 1940s, Peters [(1)][1]

We read with great interest the article by Niss et al. [(1)][1] in the March 2016 issue of iJACC. These investigators describe an abnormal mitral inflow pattern, left atrial enlargement, and pulmonary hypertension in a cohort of patients with sickle cell anemia. In the absence of a definitive

Heart failure usually occurs in the setting of a low cardiac output, however in rare cases it may be associated with a high cardiac output. We present a rare and intriguing case of a patient with end-stage renal disease (ESRD) on hemodialysis that developed high output heart failure from his arteriovenous fistula (AVF). A 36 year old African American male with ESRD secondary to hypertension, on hemodialysis for six years, presented to emergency room with a history of chest pain and shortness of breath. He was diagnosed with congestive heart failure secondary to coronary artery disease. His transthoracic echo showed an ejection fraction of 65% and both the stress test and cardiac enzymes were negative for ischemia. Further workup revealed a cardiac output of 10.6 L/min and access flow of 2.37 L/min. We made a diagnosis of high output heart failure secondary to the AVF. He was taken to surgery for a minimally invasive limited ligation endoluminal-assisted revision (MILLER) procedure after which his symptoms resolved. High output heart failure, though a rare complication of AVF placement is an important differential in hemodialysis patients who present with symptoms of congestive heart failure. An accurate diagnosis will prevent morbidity and unnecessary hospital admissions.

Although hemodialysis patients who initiate and maintain a permanent form of dialysis vascular access have improved all-cause and cardiovascular survival compared with those who use catheters, the presence of an arteriovenous fistula has been shown to have a short-term, adverse effect on cardiac function. Through its effect as a left-to-right extracardiac shunt, the arteriovenous fistula can increase cardiac workload substantially, and, in certain patients, result in a high-output state and resultant heart failure over time. Here we review the mechanisms by which dialysis arteriovenous access may promote the development of high-output cardiac failure in end-stage renal disease patients, describe risk factors for and the diagnosis of high-output heart failure, and suggest management strategies for patients who develop high-output heart failure.

Aortocava fistula is a rare condition ranging from 0.22% to 6% of all ruptured aortic aneurysms. Recognition and diagnosis of this entity can often be difficult and requires heightened clinical suspicion to ensure that prompt surgical management leads to a favorable outcome. We herein describe the diagnosis and the technical points of successful endovascular management of aortocaval fistula in the setting of a ruptured abdominal aortic aneurysm.

We report a case of a premature infant with a vein of Galen malformation (VGM) who presented with high-output heart failure, pulmonary hypertension, and respiratory distress. An echocardiogram showed normal cardiac anatomy, patent ductus arteriosus, and retrograde flow in the descending aorta. Ultrasonography and computed tomography of head confirmed the diagnosis of a large VGM. The patient developed multiorgan failure. Endovascular embolization of the VGM was successfully performed with an excellent outcome.

We thank Dr. Cerit and colleagues for their interest in our paper describing the pathophysiology and outcomes of high-output heart failure (HF) [(1)][1]. We demonstrated that obesity-related high-output HF, the most common etiology, is caused by excessive vasodilation, plasma volume expansion

We have read the paper by Martin-Perez et al. [(1)][1] with great interest. The investigators reported that high-output heart failure (HF) is an important cause of cardiac failure that is associated with increased mortality. Despite the wide variety of underlying etiologies, the causes share a

Temporary or permanent central venous catheter (CVC) insertion has been performed frequently for hemodialysis treatment. One of the most important long-term complications of CVC is the central venous occlusion (CVO). Treatment of CVO consists of percutaneous angioplasty (PTA), PTA and stent implantation, or surgical procedure for resistant occlusions. Clinical outcome and long-term results of the revascularization procedures are well documented. However, there is no clear information about acute medical complications of the procedures. High-output heart failure (HOHF) is associated with several diseases including chronic anemia, psoriasis, systemic arteriovenous fistula, sepsis, hypercapnia, multiple myeloma, and hyperthyroidism. Herein, we report a case of chronic kidney disease with CVO that developed acute HOHF immediately after the revascularization procedure (PTA and stenting).

In the hemodialysis patient population, a surgically created arteriovenous fistula is the preferred vascular access option. Development of high-output heart failure may be an underappreciated complication in patients who have undergone this procedure. When a large proportion of arterial blood is shunted from the left-sided circulation to the right-sided circulation via the fistula, the increase in preload can lead to increased cardiac output. Over time, the demands of an increased workload may lead to cardiac hypertrophy and eventual heart failure. Patients may present with the usual signs of high-output heart failure including tachycardia, elevated pulse pressure, hyperkinetic precordium, and jugular venous distension. Typically, the AV fistula is quite large and is likely located in the upper arm, more proximal to the heart. Routine access flow monitoring should demonstrate blood flows (Qa) >2000 ML/min. Echocardiogram may reveal either a low or high left ventricular ejection fraction, and right-heart catheterization demonstrates an elevated cardiac output with a low to normal systemic vascular resistance. When addressing the problem of high-output heart failure, the nephrologist is faced with the dilemma of preventing progression of heart failure at the expense of loss of vascular access. Nevertheless, treatment should be directed at correcting the underlying problem by surgical banding or ligation of the fistula.

Endovenous laser ablation treatment has become the less invasive therapeutic choice for the treatment of superficial venous insufficiency and varicose veins. A 64-year-old woman presented at our hospital with varicose veins and prior endovenous laser ablation treatment. The patient had high-output heart failure caused by a right femoral arteriovenous fistula. She was treated medically and underwent an open repair of the right superficial femoral artery and the right femoral vein with complete resolution of the arteriovenous fistula and heart failure. Here, we have reviewed the literature and discussed possible causes of the complication of arteriovenous fistula after endovenous laser ablation treatment.

We present two cases of females in their 40s presenting with biventricular heart failure being the consequence of a large arteriovenous fistula. Both patients had undergone abdominal surgery several years prior to the heart failure event with the initial finding of moderate pulmonary hypertension and high-output heart failure. CT revealed a large arteriovenous fistula between the common iliac artery and vein which subsequently was closed percutaneously.

SummaryA 68-year-old woman was admitted with dyspnea. The patient had been treated with hemodialysis for renal failure for 11 years. On admission, chest X-ray showed pulmonary edema. Right-heart catheterization revealed high cardiac output (11.8l/min) and elevated pulmonary capillary wedge pressure (PCWP). Doppler ultrasonography showed high-flow of an arteriovenous fistula (AVF) for hemodialysis. The patient was diagnosed as having high-output heart failure due to a high-flow AVF. Inflow reduction of the AVF was performed by proximal radial artery ligation. Right-heart catheterization performed 2 weeks after the operation revealed that cardiac output had decreased from 11.8 to 9.5l/min and PCWP was also reduced from 21 to 9mmHg. Furthermore, flow of the AVF measured by Doppler ultrasonography was also decreased. To our knowledge, this is the first report that assessed hemodynamics of high-output heart failure before and after inflow reduction of the AVF by repeated right-heart catheterization.

Anomalous coronary arteriovenous fistulas (CAVF) are rare and usually do not require intervention. A 75 year old female with hypertension, type 2 diabetes, chronic kidney disease, atrial fibrillation, and heart failure with preserved ejection fraction presents with three months of progressive

A 57 year-old male with Klippel-Trenaunay Syndrome (KTS) presented with four weeks of dyspnea on exertion with unilateral right lower extremity swelling and pain. Vital signs were unremarkable though exam demonstrated elevated jugular venous distension and unilateral pitting edema in the right

Hereditary hemorrhagic telangiectasia (HHT) is characterized by mucocutaneous telangiectasias and arteriovenous malformations (AVMs) leading to pulmonary and liver shunting. Liver shunts from the hepatic artery (HA) to hepatic vein (HV) cause high output heart failure, from portal vein (PV) to HV

A 40-year-old man was transferred to our hospital because of severe anasarca. He was a heavy drinker for more than 20 years, and diagnosed with diabetes mellitus 8 years earlier and treated with retinal photocoagulation 8 months earlier. He reported loss of appetite after divorce 10 months prior to admission. On admission, he presented with systemic edema and dyspnea. Chest radiography showed massive pleural effusion and cardiomegaly. Serum total protein was 5.6 g/dl, albumin 2.6 g/dl, and urinary protein excretion was 5.3 g/day. Glucose tolerance test showed normal pattern. Ultrafiltration and continuous hemofiltration resulted in loss of 40 kg body weight in 5 days. Echocardiography revealed high-output heart failure and blood tests showed low serum thiamine level of 12 ng/ml (normal, >28 ng/ml). Accordingly, the diagnosis was established as beriberi heart disease complicated with nephrotic syndrome. Treatment with 50 mg/day thiamine intravenously and 80 mg/day furosemide resulted in increase in urine output, decrease in cardiac output, resolution of pulmonary effusion, and about 70 kg body weight loss. Percutaneous renal biopsy showed nodular glomerulosclerosis, mesangial matrix expansion, and thickening of glomerular basement membrane (GBM). Immunofluorescence study showed no glomerular deposition of immunoglobulin or complement. Electron microscopy showed GBM thickening and mesangial matrix deposition without electron-dense deposits or fibrils. These findings were compatible with diabetic glomerulosclerosis. In this patient, extreme malnutrition altered glucose tolerance but, on the other hand, nephrotic syndrome associated with diabetic nephropathy made the diagnosis of beriberi heart disease difficult.

Hereditary hemorrhagic telangiectasia (HHT) is a genetic vascular disorder characterised by epistaxis, telangiectases, and visceral arteriovenous malformations. Hyperdynamic blood flow associated with arteriovenous malformations may lead to pulmonary hypertension, global heart failure, and valvular insufficiencies. We report a patient who had HHT with severe heart failure (New York Heart Association [NYHA] class III-IV) and pulmonary hypertension caused by an hepatic arteriovenous fistula. After successful transarterial embolisation of the right branch of the hepatic artery with polyvinyl alcohol (PVA) particles and coils, 4 to 7 mm in size, the patient was discharged with functional class II (NYHA) heart failure.

The symptoms and signs of heart failure can occur in the setting of an increased cardiac output and has been termed 'high output heart failure'. An elevated cardiac output with clinical heart failure is associated with several diseases including chronic anaemia, systemic arterio-venous fistulae, sepsis, hypercapnia and hyperthyroidism. The underlying primary physiological problem is of reduced systemic vascular resistance either due to arterio-venous shunting or peripheral vasodilatation. Both scenarios can lead to a fall in systemic arterial blood pressure and neurohormonal activation leading to overt clinical heart failure. In contrast to low output heart failure, clinical trial data in this area are lacking. The use of conventional therapies for heart failure, such as angiotensin converting enzyme inhibitors, angiotensin receptor blockers and certain beta-blockers with vasodilatory properties, is likely to further reduce systemic vascular resistance resulting in deterioration. The condition, although uncommon, is often associated with a potentially correctable aetiology. In the absence of a remediable cause, therapeutic options are very limited but include dietary restriction of salt and water combined with judicious use of diuretics. Vasodilators and beta-adrenoceptor positive inotropes are not recommended.

Vascular injury is a rare but not uncommon complication of spinal surgery and associated with dramatic consequences. Congestive heart failure secondary to a hyperkinetic circulation can occur in systemic diseases and in arterio-venous fistulae. A 57-year-old man was admitted to hospital complaining of dyspnoea and oedema of the left leg. Eight days previously he had undergone a discectomy at L4-L5. On auscultation a systolic-diastolic murmur was noted over the entire abdomen. An echocardiogram demonstrated an enlarged right atrium, severe mitral and tricuspid regurgitation and increased pulmonary artery pressure. An abdominal CT demonstrated irregular dilatation of the left common iliac vein and through a fistula and simultaneous opacification of the right common iliac artery; subsequently, this was also confirmed by angiography. The patient underwent an emergency endovascular stent-graft of the right common iliac artery with normalization of the venous return pressure and quick resolution of the heart failure. It is important for the physician involved in clinical work to keep in mind all the possible post-surgical complications that can occur in symptomatic patients who have recently undergone an intervention.

Wet beriberi-induced pericardial effusion has rarely been previously described. Little is known about the effect of beriberi-induced pericardial effusion on hemodynamics. Here we present a case of wet beriberi with pericardial effusion that exhibited constrictive physiology, which was dramatically improved after treatment. A 61-year-old male patient was admitted to our hospital for progressive leg edema, dyspnea on exertion, and lower-extremity muscle weakness. Echocardiography showed a hyperkinetic left ventricle and a moderate amount of pericardial effusion. Hemodynamic measurements, including simultaneous measurement of left and right ventricular pressures, revealed high output heart failure and constrictive physiology. Blood test showed lactic acidosis, and low level of serum thiamine levels; consistent with a diagnosis of wet beriberi. After thiamine replacement therapy, the patient’s hemodynamic state rapidly improved. Additionally, pericardial effusion decreased and constrictive physiology was successfully resolved. No other possible causes of pericardial effusion could be identified, with the exception of thiamine deficiency. This case illustrates the importance of considering wet beriberi as a possible cause of pericardial effusion with constrictive physiology.

Activin like kinase 1 (ALK1) mediates signaling via the TGFb family of ligands. ALK1 activity promotes endothelial proliferation and migration. Reduced ALK1 activity is associated with arteriovenous malformations. No studies have examined the effect of global ALK1 deletion on indices of cardiac remodeling. We hypothesized that reduced levels of ALK1 promote maladaptive cardiac remodeling. Methods: We employed an ALK1 conditional knockout mice (cKO) harboring the ROSA26-CreER knock-in allele whereby a single dose of intraperitoneal tamoxifen triggered ubiquitous Cre recombinase mediated excision of floxed ALK1 alleles. Tamoxifen treated wild-type (WT-Tam; n=5) and vehicle treated ALK1-cKO mice (cKO-Veh; n=5) served as controls for tamoxifen treated ALK1-cKO mice (cKO-Tam; n=15). Results: ALK1 cKO-Tam mice demonstrated reduced 14-day survival compared to cKO-Veh controls (33% vs 100%, respectively, p