Acute High-Output Heart Failure with Pulmonary Hypertension and Severe Liver Injury Caused by Amlodipine Poisoning: A Case Report.

Acute high-output heart failure (HOHF) with pulmonary hypertension and liver injury caused by amlodipine poisoning is very rare. We report a 52-year-old woman who suffered from severe shock after an overdose of amlodipine. Hemodynamic monitoring showed that while her left ventricular systolic function and cardiac output were elevated, her systemic vascular resistance decreased significantly. At the same time, the size of her right heart, her central venous pressure, and the oxygen saturation of her central venous circulation all increased abnormally. The patient's circulatory function and right ventricular dysfunction gradually improved after large doses of vasopressors and detoxification measures. However, her bilirubin and transaminase levels increased significantly on hospital day 6, with a CT scan showing patchy, low-density areas in her liver along with ascites. After liver protective treatment and plasma exchange, the patient's liver function gradually recovered. A CT scan 4 months later showed all her liver abnormalities, including ascites, had resolved. The common etiologies of HOHF were excluded in this case, and significantly reduced systemic vascular resistance caused by amlodipine overdose was thought to be the primary pathophysiological basis of HOHF. The significant increase in venous return and pulmonary blood flow is considered to be the main mechanism of right ventricular dysfunction and pulmonary hypertension. Hypoxic hepatitis caused by a combination of hepatic congestion and distributive shock may be the most important factors causing liver injury in this patient. Whether amlodipine has other mechanisms leading to HOHF and pulmonary hypertension needs to be further studied. Considering the significant increase of right heart preload, aggressive fluid resuscitation should be done very cautiously in patients with HOHF and shock secondary to amlodipine overdose.

32-year-old Traumatic Arteriovenous Fistula Presenting With Leg Ulcer and High-output Heart Failure.

Traumatic arteriovenous fistula (AVF) is not a common disorder, and dermatological signs and heart failure caused by AVF are rarely reported. We present the case of a 55-year-old woman who was referred for congestive heart failure symptoms. Echocardiography revealed preserved left ventricular ejection fraction. Due to edema of the right leg with a long-standing leg ulcer and palpable femoral thrill, duplex ultrasonography was performed. It showed an AVF between the right superficial femoral artery (SFA) and the right femoral vein (FV). The patient recalled a 32-year-old gunshot injury that was not medically treated. After the diagnosis of AVF she was referred to a surgeon for an AVF ligation, with subsequent resolution of her symptoms. The differential diagnosis of leg ulcer with leg edema should include the possibility of AVF as a cause.

A Case of High-Output Heart Failure.

CASE PRESENTATION: A 55-year-old woman with a medical history of hereditary hemorrhagic telangiectasia (HHT) complicated by recurrent nosebleeds, severe blood loss anemia, hepatic arterial-venous malformation (AVM), pulmonary hypertension, and severe tricuspid regurgitation presented to the HHT specialty clinic with acute hypoxic respiratory failure (new 3-L O2 requirement), weight gain, and volume overload. She was directly admitted to the pulmonary hypertension unit of our hospital. She had two recent admissions for similar symptoms thought to be due to worsening pulmonary arterial hypertension. In prior admissions, she had undergone right heart catheterization demonstrating mild pulmonary hypertension (pulmonary arterial pressure, 29 mm Hg, cardiac output by Fick 5.76, and cardiac index 3.22, mildly elevated pulmonary vascular resistance to 5.5 woods units). She would undergo diuresis with symptomatic improvement; however, after discharge she would rapidly develop recurrent heart failure symptoms. She reported compliance with guideline-directed medications, diuretics, and dietary restrictions and was still suffering severe symptoms. Notably she had previously elevated liver enzymes concerning for cirrhosis and had begun a workup to evaluate for causes of cirrhosis; she had a history of mild alcohol use, negative hepatitis viral serology, and no known history of liver disease.

Circulatory trajectories after out-of-hospital cardiac arrest: a prospective cohort study.

BACKGROUND: Circulatory failure frequently occurs after out-of-hospital cardiac arrest (OHCA) and is part of post-cardiac arrest syndrome (PCAS). The aim of this study was to investigate circulatory disturbances in PCAS by assessing the circulatory trajectory during treatment in the intensive care unit (ICU). METHODS: This was a prospective single-center observational cohort study of patients after OHCA. Circulation was continuously and invasively monitored from the time of admission through the following five days. Every hour, patients were classified into one of three predefined circulatory states, yielding a longitudinal sequence of states for each patient. We used sequence analysis to describe the overall circulatory development and to identify clusters of patients with similar circulatory trajectories. We used ordered logistic regression to identify predictors for cluster membership. RESULTS: Among 71 patients admitted to the ICU after OHCA during the study period, 50 were included in the study. The overall circulatory development after OHCA was two-phased. Low cardiac output (CO) and high systemic vascular resistance (SVR) characterized the initial phase, whereas high CO and low SVR characterized the later phase. Most patients were stabilized with respect to circulatory state within 72 h after cardiac arrest. We identified four clusters of circulatory trajectories. Initial shockable cardiac rhythm was associated with a favorable circulatory trajectory, whereas low base excess at admission was associated with an unfavorable circulatory trajectory. CONCLUSION: Circulatory failure after OHCA exhibits time-dependent characteristics. We identified four distinct circulatory trajectories and their characteristics. These findings may guide clinical support for circulatory failure after OHCA. TRIAL REGISTRATION: ClinicalTrials.gov: NCT02648061.

Subaortic Membranes in Patients With Hereditary Hemorrhagic Telangiectasia and Liver Vascular Malformations.

Background Patients with hereditary hemorrhagic telangiectasia have liver vascular malformations that can cause high-output cardiac failure (HOCF). Known sequelae include pulmonary hypertension, tricuspid regurgitation, and atrial fibrillation. Methods and Results The objectives of this study were to describe the clinical, echocardiographic, and hemodynamic characteristics and prognosis of hereditary hemorrhagic telangiectasia patients with HOCF who were found to have a subaortic membrane (SAoM). A retrospective observational analysis comparing patients with and without SAoM was performed. Among a cohort of patients with HOCF, 9 were found to have a SAoM in the left ventricular outflow tract by echocardiography (all female, mean age 64.8±4.0 years). The SAoM was discrete and located in the left ventricular outflow tract 1.1±0.1 cm below the aortic annular plane. It caused turbulent flow, mild obstruction (peak velocity 2.8±0.2 m/s, peak gradient 32±4 mm Hg), and no more than mild aortic insufficiency. Patients with SAoM (n=9) had higher cardiac output (12.1±1.3 versus 9.3±0.7 L/min, P=0.04) and mean pulmonary artery pressures (36±3 versus 28±2 mm Hg, P=0.03) compared with those without SAoM (n=19) during right heart catheterization. Genetic analysis revealed activin receptor-like kinase 1 mutations in each of the 8 patients with SAoM who had available test results. The presence of a SAoM was associated with a trend towards higher 5-year mortality during follow-up. Conclusions SAoM with mild obstruction occurs in patients with hereditary hemorrhagic telangiectasia and HOCF. SAoM was associated with features of more advanced HOCF and poor outcomes.

Cardiac overload resolved by resection of a large plexiform neurofibroma on both the buttocks and upper posterior thighs in a patient with neurofibromatosis type I: a case report.

BACKGROUND: A large plexiform neurofibroma in patients with neurofibromatosis type I can be life threatening due to possible massive bleeding within the lesion. Although the literature includes many reports that describe the plexiform neurofibroma size and weight or strategies for their surgical treatment, few have discussed their possible physical or mental benefits, such as reducing cardiac stress. In addition, resection of these large tumors can result in impaired wound healing, partly due to massive blood loss during surgery. CASE PRESENTATION: A 24-year-old man was diagnosed with neurofibromatosis type I and burdened with a large plexiform neurofibroma on the buttocks and upper posterior thighs. The patient was 159 cm in height and 70.0 kg in weight at the first visit. Cardiac overload was indicated by an echocardiography before surgery. His cardiac output was 5.2 L/min with mild tricuspid regurgitation. After embolism of the arteries feeding the tumor, the patient underwent surgery to remove the neurofibroma, followed by skin grafting. Follow-up echocardiography, performed 6 months after the final surgery, indicated a decreased cardiac output (3.6 L/min) with improvement of tricuspid regurgitation. Because the blood loss during the first surgery was over 3.8 L, malnutrition with albuminemia was induced and half of the skin graft did not attach. Nutritional support to improve the albuminemia produced better results following a second surgery to repair the skin wound. CONCLUSION: Cardiac overload may be latent in patients with neurofibromatosis type I with large plexiform neurofibromas. As in pregnancy, the body may compensate for this burden. In these patients, one stage total excision may improve quality of life and reduce cardiac overload. In addition, nutritional support is likely needed following a major surgery that results in either an extensive skin wound or excessive blood loss during treatment.

Early echocardiographic modifications after flow reduction by proximal radial artery ligation in patients with high-output heart failure due to high-flow forearm arteriovenous fistula.

BACKGROUND: Arteriovenous fistula (AVF) for haemodialysis (HD) induces a volume/pressure overload which impairs bi-ventricular function and increases systolic pulmonary arterial pressure (PAPS) and left ventricular mass (LVM). In the presence of high blood flow (Qa) AVF (> 1.5 L/min/1.73 m2) and cardio-pulmonary recirculation (>20%), high-output congestive heart failure (CHF) may occur and AVF flow reduction is recommended. Proximal Radial Artery Ligation (PRAL) is an effective technique for distal radio-cephalic (RC) AVF flow reduction. METHODS: we evaluated six HD and four transplant patients with high-flow RC AVF and symptoms of CHF who underwent PRAL. We compared echocardiographic (ECHO) findings before (T0) and 1 and 6 months (T1,T6) after PRAL. Preoperative ECHO was performed before (T0b) and after AVF anastomosis manual compression (T0c). RESULTS: At T1 AVF flow reduction rate was 58.4% ± 13% and 80% of patients reported improved CHF symptoms. ECHO data showed an improvement of tricuspid annular plane systolic excursion (TAPSE) at T1 (p = 0.03) and a reduction of PAPS at T6 (p = 0.04). TAPSE improved after AVF anastomosis compression during preoperative ECHO (p = 0.03). Delta of TAPSE at the dynamic manoeuvre at T0 directly correlated with early (1 month after PRAL, p = 0.01) and late (6 months after PRAL, p = 0.04) deltas of TAPSE. CONCLUSIONS: AVF flow reduction after PRAL induces immediate regression of CHF symptoms, early improvement of TAPSE and late improvement of PAPS, suggesting a prevalent right sections involvement in CHF. Preoperative TAPSE modification after AVF anastomosis compression could represent a useful evaluation tool to determine which patients would benefit of PRAL.

Intraoperative Ultrasound Guidance for Banding of an Arteriovenous Fistula Causing High Cardiac Output Heart Failure.

The creation of an arteriovenous fistula (AVF) is the preferred mode of access for hemodialysis in patients with End-Stage Renal Disease (ESRD). High output cardiac failure is a known but rare complication of AVF resulting from high flow volume. This case report describes the use of intraoperative ultrasound as a guide for the banding of an AVF to decrease flow volume in a patient with high cardiac output failure. The access was preserved, and a gradual decline of cardiac function before and recovery after banding is demonstrated over an 18-year period.

Primary External Stenting of an Autogenous Brachial-Basilic Upper Arm Transposition.

High-volume shunt flow after arteriovenous fistula (AVF) creation for hemodialysis can cause high-output heart failure. We used the Frame™ (Vascular Graft Solutions Ltd., Tel Aviv, Israel) external support, a stent, to limit vein dilatation and consecutive high-volume shunt in a 62-old female who underwent brachial-basilic upper arm transposition. After maturation, the shunt was used for dialysis and showed a plateauing flow volume 3 months after the operation. This case illustrates the safety and feasibility of this intervention when performed during AVF formation.

Infantile hepatic hemangiomas associated with high-output cardiac failure and pulmonary hypertension.

BACKGROUND: Infantile hepatic hemangioma (IHH) is a rare endothelial cell neoplasm, which may be concurrent with severe complications and result in poor outcomes. Moreover, the coexistence of IHH and congenial heart disease is even rarer. CASE PRESENTATION: We present a 10-day-old male born with IHH associated with patent ductus arteriosus (PDA), atrial septal defect (ASD) and pulmonary hypertension. Moreover, we reviewed a series of studies of IHH-associated high-output cardiac failure between 1974 and 2018, and summarized the treatment outcomes. CONCLUSIONS: Infantile hepatic hemangioma (IHH) has been known to induce high-output heart failure. There is no literature to summarize the severity of its impact on heart, which can lead to a high mortality rate. When IHH is detected by ultrasound, the heart should be evaluated to facilitate treatment. The outcomes of IHH associated with heart failure are good.

Features of high flow access in the elderly.

INTRODUCTION: The number of elderly hemodialysis patients continues to grow. The aim of this study was to investigate differences in elderly high flow access patients compared with middle-aged and young patients. METHODS: We performed a retrospective study to determine the characteristics of elderly patients (aged >60 years) following blood flow suppression procedures. Preoperative and postoperative data from 177 patients who underwent blood flow suppression procedures and 73 patients who underwent procedures for run-off vein ligation and subcutaneous fixation of the superficial artery were compared. RESULTS: A high proportion of young (aged 20-40 years) and middle-aged (aged 41-60 years) patients met the criteria for blood flow suppression procedures (flow volume 1500 mL/min, flow volume/cardiac output 35%), whereas a high proportion of elderly patients did not. Moreover, heart strain could evidently be caused even with low flow volume. In elderly patients, a tricuspid regurgitation pressure gradient and right heart strain were observed more frequently. CONCLUSION: Elderly patients who underwent blood flow suppression procedures or subcutaneous fixation of the superficial artery exhibited lower flow volume, and the effects of high flow access in elderly patients depend on the nature of vascular changes. Ultimately, the underlying conditions and hemodynamics of each patient must be determined on an individual basis.

Relationships of high cardiac output with ventricular morphology, myocardial energetics, and energy costs in hemodialysis patients with preserved ejection fraction.

Hemodialysis patients have conditions that increase cardiac output (CO), including arteriovenous fistula, fluid retention, vasodilator use, and anemia. We sought to determine the relationships between these factors and CO and to evaluate the effects of the high-output states on ventricular morphology, function, and myocardial energetics in hemodialysis patients, using noninvasive load-insensitive indices. Cardiovascular function was assessed in hemodialysis patients with high output [ejection fraction ≥ 50%, cardiac index (CI) > 3.5 L/min/m2, n = 30], those with normal output (CI < 3.0 L/min/m2, n = 161), and control subjects without hemodialysis (n = 155). As compared to control subjects and hemodialysis patients with normal CI, patients with elevated CI were anemic and displayed decreased systemic vascular resistance index (SVRI), excessive left ventricular (LV) contractility, larger LV volume, and tachycardia. Lower hemoglobin levels were correlated with decreased SVRI, excessive LV contractility, and higher heart rate, while estimated plasma volume and interdialytic weight gain were associated with larger LV volume, thus increasing CO. High output patients displayed markedly increased pressure-volume area (PVA) and PVA/stroke volume ratio, which were correlated directly with CO. The use of combination vasodilator therapy (angiotensin-converting enzyme inhibitor/angiotensin-receptor blocker and calcium channel blocker) was not associated with high-output states. In conclusion, anemia and fluid retention are correlated with increased CO in hemodialysis patients. The high-output state is also associated with excessive myocardial work and energy cost.

Liver Transplantation Trends and Outcomes for Hereditary Hemorrhagic Telangiectasia in the United States.

BACKGROUND: Liver arteriovenous malformations (AVM) in hereditary hemorrhagic telangiectasia (HHT) can necessitate liver transplantation. There is limited data on HHT patients undergoing liver transplantation (LT) in the United States. METHODS: Two sources of data were used: (1) Scientific Registry of Transplant Recipients (SRTR) database (1998-2016) (2) Single center liver transplant database (Mayo Clinic Rochester, MN). The aims of this study were (1) to determine trends in LT for HHT-related liver involvement in the United States using the SRTR database; (2) to identify clinical characteristics, indications, and outcomes for LT in HHT. RESULTS: Thirty-nine HHT patients were listed for LT in the SRTR database from 1998-2016 to 1998-2001 (n = 1); 2002-2005 (n = 4); 2006-2010 (n = 10), and 2011-2016 (n = 24). Twenty-four underwent LT at a median age of 47.5 years (interquartile range, 37.0-58.5 years). Median calculated MELD score at time of LT was 8.0 (interquartile range, 7.0-9.5), and 75% received an exception MELD score. Two status-1 patients died during transplant surgery. Nineteen (86%) patients were alive after a median post-LT follow-up of 48 months, whereas 2 patients were lost to follow-up. Five of the aforementioned HHT patients underwent LT at Mayo Clinic, 4 with high output cardiac failure, and 1 with biliary ischemia. All 5 were alive at the time of last follow-up with good graft function and resolution of heart failure. CONCLUSIONS: Outcomes after LT for HHT patients are excellent with 86% survival after a median follow-up of 48 months and resolution of heart failure. LT listing for HHT has increased in substantially in more recent eras.

High-flow arteriovenous fistula and heart failure: could the indexation of blood flow rate and echocardiography have a role in the identification of patients at higher risk?

BACKGROUND: Although only high-flow arteriovenous fistulas (AVFs) are postulated to cause high-output cardiac failure (HOCF), there are currently no universally accepted criteria defining a high-flow fistula. METHODS: To verify if vascular access blood flow (Qa) ≥ 2000 ml/min provides an accurate definition of high-flow fistula, we selected 29 consecutive patients with Qa ≥ 2000 ml/min at color-duplex ultrasound examination and assessed them for the presence of cardiac failure symptoms; transthoracic echocardiography was also performed. RESULTS: Nineteen patients (65%) had heart failure symptoms and were classified with HOCF. At receiver operating characteristic (ROC) curve analysis, Qa ml/min values did not identify patients with heart failure symptoms but when AVF blood flow was indexed for height2.7, Qa ≥ 603 ml/min/m2.7 detected the occurrence of HOCF with good accuracy (sensitivity 100%, specificity 60%, efficiency 86%, positive predictive value 83%, negative predictive value 100%, area under curve 0.75). At echocardiographic evaluation, patients with Qa ≥ 603 ml/min/m2.7 had a more severe increase of left ventricular mass (63 ± 18 vs. 47 ± 7 g/m2.7, p < 0.003), left ventricular diastolic volume (140 ± 42 vs. 109 ± 14 ml, p < 0.007), left atrial volume (53 ± 23 vs. 39 ± 5 ml/m2, p < 0.015), a higher incidence of diastolic dysfunction (70 vs. 17%, p < 0.019) and higher CO reduction after AVF manual compression (2151 ± 875 vs. 1292 ± 527 ml/min, p < 0.009) than patients with Qa < 603 ml/min/m2.7. CONCLUSIONS: Indexation of AVF blood flow should be considered in defining high-flow fistula because the effect of Qa may differ in individuals of different sizes. A Qa value ≥ 603 ml/min/m2.7 and its association with some echocardiographic alterations could identify patients at higher risk for HOCF.

Endovascular Repair of Iatrogenic Iliocaval Fistula Causing High-Output Cardiac Failure after Spine Fusion.

The case being presented is a 35-year-old female with a 3-year history of progressive dyspnea and right-sided heart failure following spine surgery. Physical examination identified a continuous bruit in the lower abdomen radiating to her back which prompted further evaluation. Echocardiography showed normal left ventricle systolic function, enlarged right ventricle, functional tricuspid regurgitation, and moderate pulmonary hypertension. A computed tomography (CT) scan of the abdomen and pelvis demonstrated findings consistent with an arteriovenous fistula (AVF) between the right common iliac artery and the inferior vena cava. She underwent an uneventful endovascular repair without perioperative complication. The patient's symptoms resolved a few hours after the procedure and she continued to be symptom free at 3-month follow-up. This case illustrates an iatrogenic iliocaval fistula causing high-output cardiac failure which was successfully treated endovascularly with excellent clinical result.

Improved Diastolic Function Is Associated With Higher Cardiac Output in Patients With Heart Failure Irrespective of Left Ventricular Ejection Fraction.

BACKGROUND: Little is known regarding the impact of diastolic function on cardiac output (CO) in patients with heart failure, particularly in patients with lower ejection fraction. This study aimed to evaluate the impact of end-diastolic pressure-volume relationship (EDPVR) on CO and end-diastolic pressure (EDP). METHODS AND RESULTS: We retrospectively analyzed 1840 consecutive patients who underwent heart catheterization. We divided patients into 8 groups according to ejection fraction (EF) (35-45%, 46-55%, 56-65%, and 66-75%) and EDP (>16 or ≤16 mm Hg). We estimated EDPVR from single measurements in the catheterization data set. Then, we replaced EDPVRs of high-EDP groups with those of normal-EDP groups and compared CO before and after EDPVR replacement. Normalized EDPVR significantly increased CO at EDP=10 mm Hg regardless of EF (EF 35-45%, from 4.5±1.6 to 4.9±1.0; EF 46-55%, 4.6±1.3 to 5.1±1.1; EF 56-65%, 4.9±1.5 to 5.2±1.0; EF 66-75%, 4.9±1.5 to 5.2±1.1). Changes in CO were similar across EF groups. CONCLUSIONS: Diastolic function normalization was associated with higher CO irrespective of EF. Diastolic dysfunction plays an important role in determining CO irrespective of EF in heart failure patients.

Right Ventricular Enlargement within Months of Arteriovenous Fistula Creation in 2 Hemodialysis Patients.

Surgically created arteriovenous fistulae (AVF) for hemodialysis can contribute to hemodynamic changes. We describe the cases of 2 male patients in whom new right ventricular enlargement developed after an AVF was created for hemodialysis. Patient 1 sustained high-output heart failure solely attributable to the AVF. After AVF banding and subsequent ligation, his heart failure and right ventricular enlargement resolved. In Patient 2, the AVF contributed to new-onset right ventricular enlargement, heart failure, and ascites. His severe pulmonary hypertension was caused by diastolic heart failure, diabetes mellitus, and obstructive sleep apnea. His right ventricular enlargement and heart failure symptoms did not improve after AVF ligation. We think that our report is the first to specifically correlate the echocardiographic finding of right ventricular enlargement with AVF sequelae. Clinicians who treat end-stage renal disease patients should be aware of this potential sequela of AVF creation, particularly in the upper arm. We recommend obtaining preoperative echocardiograms in all patients who will undergo upper-arm AVF creation, so that comparisons can be made postoperatively. Alternative consideration should be given to creating the AVF in the radial artery, because of less shunting and therefore less potential for right-sided heart failure and pulmonary hypertension. A multidisciplinary approach is optimal when selecting patients for AVF banding or ligation.