High-output Failure Research Articles

P1354POSTOPERATIVE MATURATION AND CHANGES IN CARDIAC FUNCTION DIFFER BETWEEN TYPES OF VASCULAR ACCESS

Abstract Background and Aims The blood flow of vascular access can be increased about tens of times after access creation. If the flow of vascular access is too low, vascular access does not provide sufficient circulation for hemodialysis and sometimes gets thrombosed. However, if the flow of vascular access is too high, it can result in high cardiac output and trigger heart failure. Although patients receiving hemodialysis require vascular access with necessary and sufficient flow, the actual change in flow after access creation is not well known. We investigated the relationships among preoperative factors, types of vascular access, clinical courses of access maturation, and changes in cardiac function. Method This single-center retrospective observational study included patients who underwent vascular access-related surgery, except thrombectomy, in 2016. Diameters and flow volumes of the brachial artery were examined using Doppler ultrasound before surgery and 1 week, 12 months, and 24 months after surgery. Cardiac functions were assessed using sonography at the same timepoints. Patients’ background information and data related to surgery, such as anastomosis size, were extracted from medical records. The obtained data were statistically analyzed. Results Fifty-eight patients [37 arteriovenous fistula (AVF), 10 arteriovenous graft (AVG), and 11 partial replacement using grafts (PR)] participated in this study. Diameters of the brachial artery increased from 4.7 mm to 5.4 mm at 1 week after access surgery. Blood flows of the brachial artery also increased from 106 mL/min to 699 mL/min. Blood flow through AVG at 1week was significantly higher than that through AVF (940 ml/min vs. 589 ml/min). Although blood flow through AVF at 12 and 24 months after access creation was significantly increased than that at 1 week after access creation, blood flow through AVG at 12 and 24 months after access creation did not show significant changes, and blood flow through PR at 12 and 24 months after access creation was significantly decreased. AVG and PR required more catheter intervention for vascular access than AVF during this observational period. Preoperative blood flow of the brachial artery and cardiac outputs were positively correlated to postoperative blood flow through AVF. However, this relationship was not observed in AVG and PR cases. The amount of cardiac output increased from 4.2 L/min before surgery to 4.4 L/min and 4.6 L/min at 12 and 24 months, respectively, although not significantly. Cardiac output at 24 months after surgery significantly increased only in AVF cases. Conclusion The clinical maturation course after vascular access creation surgery differs between AVF and AVG cases. Because blood flow through AVF is likely to increase gradually after access creation, surgeons should consider cardiac stresses related to vascular access, especially in cases with high-flow brachial artery or high cardiac output before surgery. Blood flow through AVG is not likely to increase in the long course. However, because blood flow through AVG just after access creation is high, regardless of the brachial artery size, surgeons should consider the risk of arteriovenous access-related ischemic steal syndrome after surgery especially in cases with severe arteriosclerosis. PR is not likely to affect blood flow volume of accesses and cardiac function.

Embolization Techniques for Arteriovenous Malformations in Parkes-Weber Syndrome

Multiple high-flow arteriovenous malformations (AVMs) are the hallmark of Parkes-Weber syndrome (PWS). Surgical resection has historically shown poor outcomes with PWS. The aim of this study was to assess the management of PWS in the current era. A retrospective review was performed from 2004 to 2017 on all patients presenting to a single institution for the management of PWS. Presentation, techniques, complications, and outcomes were reviewed. Fourteen patients (50% male) with PWS were seen at our institution, and mean age was 19.9years (range, 4.7-68.8). The lower extremity was affected in 12 (86%) and the upper extremity in 2 (14%) patients. All patients presented with pain and swelling in the affected limb. Seven (50%) patients presented with ulcers, of which 3 (43%) had extensive wounds. Five (36%) patients had echocardiographic evidence of high-output cardiac failure. All patients underwent angiography with the intention to treat. Three (21%) patients were found to have diffuse arteriovenous communication with no discrete AVM nidus and thus did not undergo intervention. The remaining 11 (79%) patients underwent transcatheter embolization of the AVM's arterial inflow. Six (55%) patients required multiple or staged inflow embolization procedures, with a mean of 3.3 (range, 1-10) interventions. Thirty-two arterial embolization procedures were performed in total. n-Butyl-2-cyanoacrylate (nBCA) adhesive was used in 22 (69%), microspheres in 8 (25%), and a combination of coils and nBCA adhesive in 2 (6%) cases. Technical angiographic success was seen in all patients. Six (55%) patients also had interventions to treat the venous component of the malformation, either concomitantly or during a separate procedure. This included radiofrequency ablation in 1 (17%), coil embolization in 1 (17%), sodium tetradecyl sulfate (STS) sclerotherapy in 2 (33%), and a combination of STS, coil embolization, and vein stripping in 2 (33%) patients. Ten (91%) patients experienced a partial response and 1 (9%) patient experienced no response to treatment. No patients had a complete response, as expected with the diffuse nature of this disease. There were no periprocedural complications. Two of 3 patients with complex wounds required major amputations for gangrene, including one above-knee and one below-knee amputation at 128months and 66months after the index procedure, respectively. AVMs in PWS can be successfully treated by a transcatheter approach. Multiple interventions are usually required. Patients with extensive wounds remain at risk for loss of limb.

Short interposition with a small-diameter prosthetic graft for flow reduction of a high-flow arteriovenous fistula

ObjectiveThe objective of this study was to evaluate the outcome of a short interposition using a small-diameter prosthetic graft as a flow-limiting procedure to manage symptomatic high-flow arteriovenous fistula (AVF). MethodsA retrospective review of medical records on a case series was conducted. From June 2004 to April 2017, there were 25 patients with clinical symptoms of high output cardiac failure and progressive dilation of aneurysmal fistula vein due to high-flow AVF (≥1.5 L/min) who underwent short interposition with a 5-mm prosthetic graft at Saitama Medical Center. The primary outcome was the relief of clinical symptoms; other outcome measures included technical success, surgical complications, patency of vascular access, and postoperative changes in local and systemic hemodynamics as assessed by Doppler ultrasound. ResultsTwenty-five patients underwent short interposition for cardiac indications (n = 16) and aneurysmal dilation (n = 9). The technical success rate was 100%. The clinical symptoms were relieved in 24 patients (96.0%). Mean reduction in access blood flow was 52.4%. Cumulative primary unassisted patency rates (± standard error) at 1 year, 2 years, and 3 years were 76.2% ± 9.3%, 70.4% ± 10.3%, and 58.1% ± 11.6%, respectively. Secondary patency rates (± standard error) at 1 year, 2 years, and 3 years were 81.8% ± 8.2%, 71.5% ± 9.9%, and 71.5% ± 9.9%, respectively. Complications included access occlusion due to late thrombosis (n = 5 [21.7%]) and graft infection (n = 1 [4.3%]) in the median follow-up period of 3.9 years. ConclusionsShort interposition with a prosthetic graft is a simple, effective, and durable treatment option for end-stage renal disease patients with cardiac symptoms and progressive dilation of the fistula vein due to high-flow AVF, offering clinical symptom resolution while preserving the autologous behavior of the initial access.

SAT-508 Oh My Thyrotoxic Heart! An Uncommon Presentation of High Output Heart Failure

High output heart failure is an extremely rare manifestation of thyrotoxicosis in patients without heart disease. Thyroid hormone has a potent metabolic, vascular, positive chronotropic and inotropic effects. Thyrotoxicosis is accompanied by a reduction in systemic vascular resistance and an obligatory increase in cardiac output to compensate high metabolic state. Most of the patients had completed or near completed recovery of cardiac function after treatment to euthyroid status, if cut in time.Case of 70 y/o female with past medical history of hypothyroidism, hypertension and chronic smoker, came to emergency room due to lightheadedness, weakness, shortness of breath, palpitations and acute bedridden for one week ago. Denied fever, nausea, vomiting or chest pain. On examination patient presented with 122bpm, 23rpm and normotensive. She was in acute respiratory distress requiring BiPAP. Lungs auscultation bilateral crackles, decrease sounds in the bases. Cardiac exam significant for tachycardia, lower extremity edema and JVD +. Laboratories significant for CKD stage 2, chronic anemia and hypoxemia. EKG no ST changes neither T depressions. CXR day #1 reported no cardiopulmonary disease. Basing in these she was admitted with Suspected Pulmonary Embolism. Well’s score was moderate risk; a Chest CTA and full anticoagulation were ordered. Later, venous duplex reported no DVT and D-dimer levels 1.65μg/ml. TSH levels significant decrease 0.0002mU/L and T4 1.800ng/dL. CXR Day#2 reported pulmonary edema and pleural effusion. ECHO2D moderate LVH and preserved systolic function. Patient referred her doctor recently increased thyroid medications from 50 to 100 mcg. Instead of presenting with pulmonary embolism, she has hyperthyroidal state causing cardiac failure. Levothyroxine, heparin and Chest CTA scan were cancelled. She was started in Atenolol and diuretics. Patient symptoms improved and was discharged home to be followed in the clinic. After one week, TSH levels were in 0.008mU/L and one month later in 3.032mU/L. She was started in Levothyroxine 50mcg, to maintain patient euthyroid state.This case illustrates that sometimes tachycardia and tachypnea are symptoms which frequently presents as baffling diagnostic problems. The association of thyrotoxicosis and cardiovascular morbidity is well established. Thyrotoxicosis most common cardiac manifestation is high output heart failure. Patients presenting with heart failure may have thyrotoxicosis as the underlying cause. Treatment of the thyrotoxicosis can restore normal heart function. Hyperthyroidal state may be take into consideration as a differential of tachycardia and tachypnea even if it’s not one of the common causes. Awareness of this presentation may help identify patients with reversible dilated cardiomyopathy and other complications.

SAT-459 A Grave Complication: Pulmonary Arterial Hypertension

There is currently emerging evidence that thyroid disease can have an impact on pulmonary pathologies but a direct causation, as opposed to correlation, is yet to be established. We present a unique case of newly diagnosed Graves’ disease with pulmonary sequalae.69 year old non-smoking female with no known medical history presented to the hospital with acute onset of chest tightness, shortness of breath and palpitations. She had been experiencing night sweats and 10 lbs weight loss in the 3 weeks preceding hospitalization. She did not endorse any pertinent family history. On exam, she presented with tachycardia. The rest of the physical exam remained unchanged. Complete blood count and comprehensive metabolic panel did not reveal any abnormalities. Her TSH was suppressed below lab capability of measurement (<0.015). Free T4 was 5.97 ng/dL, and total T3 was 492 ng/dL. Subsequently obtained TPO antibody level was > 1000 IU/ml and TSI was 29.3 IU/L. EKG showed atrial fibrillation with RVR. Thyroid U/S showed that the thyroid gland was diffusely enlarged to the upper limit of normal but no nodules. CT chest showed diffuse ground glass opacities.She was treated with propranolol, and methimazole after resolution of acute symptoms. She was placed on diuretics to treat symptoms of pulmonary edema secondary to uncontrolled atrial fibrillation and high output heart failure. On outpatient follow up she continued to have mild shortness of breath on exertion. She had normal systolic and diastolic function of heart but an elevated PAP of 38mm Hg on a transthoracic echocardiogram, and her pulmonary function tests were within normal limits for her age. A follow up CT chest, 6 weeks after discharge, showed no resolution of ground glass opacities.Current literature shows that there might be pulmonary effects of hyper-thyroidal diseases; such as ventilation disorders, OSA, CSA, and pleural effusions. A 2002 study revealed a strong association of hyperthyroidism with pulmonary hypertension. A 2016 literature review established that there is specifically an association of Graves’ disease with pulmonary arterial hypertension. Both of these studies have had a small patient group. Our case perhaps adds credence to the fairly less known affiliation of hyperthyroidism with pulmonary disease. A unique finding in our patient - unresolving ground glass opacities, suggests a possible thyroid related interstitial lung disease. As with our case, there needs to be an increased awareness in the among clinicians to further work up both thyroid related pulmonary hypertension and interstitial lung disease possibly related to uncontrolled thyroid pathology. Increased vigilance to include hyperthyroidism as a differential for pulmonary hypertension can lead to better clinical outcomes for patients, with less possible invasive and expensive testing of other causes of pulmonary hypertension.

SUN-513 Severe Hyperthyroidism in a Complete Molar Pregnancy

Hydatidiform mole (HM), a type of gestational trophoblastic disease (GTD), is a rare cause of clinical hyperthyroidism. The development of hyperthyroidism requires an elevation of HCG >100,000 mlU/mL for several weeks. Complete mole has a marked HCG elevation compared to partial mole thus presents with a higher incidence of thyrotoxicosis. Surgical uterine evacuation is the treatment of choice for HM. However, untreated hyperthyroidism can pose a risk for the development of thyroid storm and high-output cardiac failure in the perioperative period. To our knowledge, there are no specific guidelines for management at this time. We present a case of hyperthyroidism secondary to complete molar pregnancy successfully treated with propylthiouracil (PTU), potassium iodide (SSKI), and atenolol in the preoperative period.A 42-year-old female with history of migraines presented to her gynecologist with a 3-week history of lower abdominal cramping, vomiting, loss of appetite, and abnormal vaginal bleeding. She also endorsed a 6-pound weight loss, intermittent tachycardia, exertional dyspnea, and increased anxiety. Pregnancy test was positive, and ultrasound was concerning for GTD. Laboratory work up was significant for HCG 797,747 mIU/mL (< 5mlU/mL), TSH <0.005 mIU/mL (0.4-4.0 mlU/mL), Free T4 3.09 ng/dL (0.9-1.9 ng/dL), and Free T3 11.48 pg/dL (1.76-3.78 pg/dL). The patient was admitted to the hospital and started on PTU 100 mg Q6H, SSKI 200 mg TID following the first dose of PTU, and atenolol 25 mg daily. She underwent an uncomplicated D & C the next day. On post-op day 1, HCG decreased to 195,338 mIU/mL and Free T4 to 2.39 ng/dL. The patient was discharged on the aforementioned doses of PTU and atenolol. One-week follow-up labs showed HCG 8,917 mIU/mL and Free T4 1.22 ng/dL. Surgical pathology confirmed a complete hydatidiform mole. PTU was decreased to 50 mg TID. On post-op day 14, HCG had risen to 15,395 mIU/mL with onset of nausea and vomiting. Repeat Free T4 remained within reference range. Patient was taken back to surgery for a laparoscopic total hysterectomy with bilateral salpingectomy. Pathology confirmed an invasive hydatidiform mole. Two-week follow-up lab work showed HCG 155 mIU/mL, TSH 1.5 mIU/mL, and Free T4 1.19 ng/dL. PTU and atenolol were then discontinued.The development of hyperthyroidism in molar pregnancy is largely influenced by the level of HCG and usually resolves with treatment of GTD (1). However, it’s crucial to control thyrotoxicosis to avoid perioperative complications. This case also highlights the importance of monitoring HCG levels following a complete molar pregnancy due to an increased risk for invasive neoplasm.1. Walkington, L et al. “Hyperthyroidism and human chorionic gonadotrophin production in gestational trophoblastic disease.” British journal of cancer vol. 104,11 (2011): 1665-9. doi:10.1038/bjc.2011.139

The Clinical Relevance and Management of High-Flow Arteriovenous Access.

Obtaining an arteriovenous (AV) access for initiation of hemodialysis is considered the gold standard as it reduces risks of infections, hospitalizations, and need for interventions. It is well documented that creation of AV access can cause or aggravate heart failure. Once AV access is created, blood volume, cardiac contractility, and left ventricular end-diastolic volume increase in a nonphysiologic fashion resulting in an overall increase in cardiac output. Left ventricular hypertrophy, diastolic dysfunction, pulmonary hypertension, and high-output cardiac failure can occur. AV accesses that have blood flows greater than 1.5L per minute are of high risk. When access flow exceeds 25% to 30% of cardiac output, the risk of developing high-output heart failure increases. Studies suggest that a blood flow (Qa)/cardiac output (CO) ratio of greater than 0.30 should be used as a screening tool to perform further cardiac testing. Depending on the severity of symptoms, management can range from banding procedure (flow reduction) or need for total abandonment of the AV access. The complications associated with high-flow AV accesses are often overlooked. Nephrologists and vascular access experts should work in tandem to mitigate potential harm to patients on dialysis who are afflicted by this condition.

Evaluation of cardiac dysfunction in adult zebrafish using high frequency echocardiography

AimsRecently, the zebrafish has gained attention as an innovative experimental model to decipher molecular and cellular mechanisms involved in cardiovascular development and diseases. Nevertheless, the use of zebrafish models has been challenged because the transparency of these fish, which allows for accurate cardiac evaluation, disappears in adulthood. In this study, the epicardial outline method was performed to investigate the feasibility of echocardiography in assessing cardiac function in pathological adult zebrafish. Materials and methodsWe attempted to estimate heart failure in adult zebrafish treated with three distinct regulators of cardiac function: phenylhydrazine hydrochloride (PHZ), doxorubicin (DOX), and ethanol. B-mode and Doppler images were evaluated at frequencies of up to 50 MHz and 40 MHz, respectively. The correlation between alterations in cardiac function, haemoglobin concentration, and myocardial histopathology were assessed. Key findingsCardiac output (CO) in PHZ-treated zebrafish was significantly higher than that in control zebrafish (151 ± 67 vs. 84 ± 37 μl/min, P = 0.004), whereas ejection fraction (EF) was lower (36.3 ± 10.9 vs. 50.9 ± 8.7%, P < 0.001), indicating typical high output heart failure derived from anaemia. Additionally, ventricular dysfunction in DOX-treated zebrafish was characterised by low CO (57 ± 38 μl/min) and EF (28.8 ± 10.4%), accompanied by an enlarged ventricle in diastole and systole, representing low output heart failure. For ethanol-treated zebrafish, EF was markedly reduced (39.6 ± 7.2%) indicating a dilated heart, while CO remained unchanged (90 ± 40 μl/min). SignificanceThe epicardial outline method is an effective way of using echocardiography to assess cardiac dysfunction in pathological adult zebrafish, unlocking a major bottleneck in this research field with limited cardiac functional assays.

Cardiac remodeling and arrhythmogenesis are ameliorated by administration of Cx43 mimetic peptide Gap27 in heart failure rats

Alterations in connexins and specifically in 43 isoform (Cx43) in the heart have been associated with a high incidence of arrhythmogenesis and sudden death in several cardiac diseases. We propose to determine salutary effect of Cx43 mimetic peptide Gap27 in the progression of heart failure. High-output heart failure was induced by volume overload using the arterio-venous fistula model (AV-Shunt) in adult male rats. Four weeks after AV-Shunt surgery, the Cx43 mimetic peptide Gap27 or scrambled peptide, were administered via osmotic minipumps (AV-ShuntGap27 or AV-ShuntScr) for 4 weeks. Cardiac volumes, arrhythmias, function and remodeling were determined at 8 weeks after AV-Shunt surgeries. At 8th week, AV-ShuntGap27 showed a marked decrease in the progression of cardiac deterioration and showed a significant improvement in cardiac functions measured by intraventricular pressure-volume loops. Furthermore, AV-ShuntGap27 showed less cardiac arrhythmogenesis and cardiac hypertrophy index compared to AV-ShuntScr. Gap27 treatment results in no change Cx43 expression in the heart of AV-Shunt rats. Our results strongly suggest that Cx43 play a pivotal role in the progression of cardiac dysfunction and arrhythmogenesis in high-output heart failure; furthermore, support the use of Cx43 mimetic peptide Gap27 as an effective therapeutic tool to reduce the progression of cardiac dysfunction in high-output heart failure.

Large Malignant Pheochromocytoma Causing Cardiac Failure and Metastasis: A Case Report With Review of Literature

Extra-adrenal functional neuroendocrine neoplasms are termed paragangliomas. We describe a case of a large abdominal neuroendocrine tumor that was proved to be a paraganglioma on biopsy. Unfortunately, the patient presented with high output heart failure, an extremely rare complication of paraganglioma. Prior to surgical biopsy, the patient underwent a complete invasive and non-invasive cardiac workup, in addition to biochemical testing. On a PET CT, the retroperitoneal neuroendocrine tumor was shown to have been metastasized to the spine. Chemotherapy targeted at somatostatin analogs was initiated. The unique presentation and rare complications are presented with relevant review of literature to highlight the spectrum of disease geared towards diagnostic and interventional radiologists. It is crucial to understand the broad spectrum of disease and include this entity in the differential diagnosis of a retroperitoneal mass. Equally significant is to consider potential complications such as metastases and cardiac failure to guide appropriate workup and management.

Short- and mid-term effects of covered stent implantation on extremity findings and heart failure in Parkes Weber syndrome: a case report.

Background Parkes Weber syndrome (PWS) is a congenital disease characterized by vascular malformations, such as arteriovenous fistulas (AVFs). It frequently presents with overgrowth of a lower limb and high-output heart failure. The main treatment is to close vascular malformations. Surgical excision or endovascular coil insertion was performed in a few patients with AVFs. However, vascular covered stent implantation has not been used for treating PWS.Case summary A 15-year-old male patient with PWS presented to our hospital because of dyspnoea and massive left upper limb swelling. After initial examination and left upper limb angiography, his symptoms and findings were attributed to the presence of high-flow large AVFs despite the presence of many coils previously inserted. We decided to implant a covered stent along the AVFs between the subclavian and axillary arteries. After stent implantation, the patient’s complaints and findings improved during the early term but they relapsed at the 6th month after percutaneous intervention.Discussion Here, we report for the first time the use of covered stent implantation and its short and 6 months results in a patient with PWS. Although initial improvements were noted, the clinical outcome at 6 months after stent implantation was poor. This was probably associated with the presence of widespread subtle AVFs or collateral connections among the existing AVFs. Based on our result, we propose that closure of large AVFs is not useful and more definitive interventions, such as limb amputation may be required earlier.

SHOSHIN BERIBERI PRESENTING AS NEW ONSET SYSTOLIC HEART FAILURE AND REFRACTORY HYPOTENSION

Patients with severe thiamine deficiency leading to wet beriberi typically present with high output heart failure and predominately right sided symptoms, but a rare and acute form of beriberi known as Shoshin Beriberi can lead to acute cardiovascular collapse. A 52-year-old woman with a history of

LOOKING BEYOND THE HEART: A VERY UNUSUAL CAUSE OF HEART FAILURE IN A YOUNG HEALTHY FEMALE

High output heart failure is a less frequent cause of heart failure and numerous etiologies should be considered in order to evaluate and treat these patients. A 32-year-old female presented with dyspnea for 1 week. Physical exam revealed bilateral lung crackles, normal S1, S2, and a faint S3, and

Femoral arteriovenous fistula associated with surgery of proximal femoral fracture: a systematic review of the literature and case presentation.

BackgroundProximal femoral fracture (PFF), such as intertrochanteric femoral fracture or femur neck fracture, and its management are crucial issues to surgeons. PFF has been dramatically is becoming exponentially prevalent, and it is at high risk of complication and mortality because it is frequently associated with serious trauma and advanced age, especially in patients treated with anticoagulants or antiplatelet agents. Surgical management is essential for the treatment of PFF. Unfortunately, current surgical procedures have been related to accompanied by vascular complications, including laceration, hemorrhage, thrombosis, embolism, intimal flap tear and pseudoaneurysm. Furthermore, these vascular injuries following surgical management of PFF are potentially limb- and life-threatening. Of the complications after operation of PFF, femoral arteriovenous fistula (AVF) is rare, but remains a challenging problem because it is frequently associated with significantly high mortality and morbidity and is very difficult to treat.MethodsA systematic literature review was conducted using the PRISMA guidelines with no language restriction. We searched scientific publications via PubMed, Embase, Cochrane central register of controlled trial, Google Scholar, the KoreaMed and the Research Information Sharing Service database. The goal of this study was to report on the incidence, clinical presentation, diagnosis, treatment, associated complications, morbidity and mortality of femoral AVF caused by PFF and to draw special attention to its prevention and management.ResultsA total of 7 case reports on femoral AVF associated with operation of PFF were identified, and one our case was added to the systematic analysis. Of the 8 cases, 4 were male and 4 were female under the age of 67.87±18.44; 6 (75.0%) survived without any events, 1 (12.5%) survived with a sequela of peroneal nerve impairment, and 1 (12.5%) died of multi-organ failure and hypovolemia.ConclusionsThe incidence of femoral AVF associated with PFF is extremely low, though it appears to increase with the rising frequency of PFF. With a very few exceptions, complications following internal fixation are potentially limb- and life-threatening. There is still no definite consensus on the standardized diagnostic or therapeutic modalities. Therefore, surgeons should keep in mind that this serious complication requires early diagnosis and prompt treatment, which should not be underestimated. Femoral AVF following operation of PFF should be meticulously managed, because untreated fistulae result in serious unexpected complications including renin-mediated hypertension, high-output heart failure and venous and/or arterial insufficiency. Surgical treatment is still the gold standard for such cases, but in limited cases endovascular procedures using embolization and closure device can be a good treatment option.

A CASE OF MULTIPLE MYELOMA DISGUISED AS HEART FAILURE

High output heart failure is an infrequently encountered entity characterized by normal cardiac function with low systemic vascular resistance. It's defined as cardiac output more that 8L/min of cardiac index more than 4.0/min/m2. It is due to an underlying disease process leading to increased

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.

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. 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). 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. 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.

Bevacizumab for treating Hereditary Hemorrhagic Telangiectasia patients with severe hepatic involvement or refractory anemia.

ObjectiveTo report our clinical experience with bevacizumab in a cohort of Hereditary Hemorrhagic Telangiectasia (HHT) patients with severe hepatic involvement and/or refractory anemia.MethodsObservational, ambispective study of the Institutional Registry of HHT at Hospital Italiano de Buenos Aires. Patients were treated with bevacizumab due to iron deficiency refractory anemia secondary to nasal/gastrointestinal bleeding and/or high output cardiac failure. We describe basal clinical data, bevacizumab schedules, efficacy outcomes and adverse events. Wilcoxon signed ranks test and longitudinal analysis were conducted.ResultsTwenty adult patients were included from July 2013 to June 2019. Clinical indications were: 13 for anemia, 4 for heart failure and 3 for both. In the anemia group, median pretreatment hemoglobin was 8.1 g/dl [IQR: 7.2–8.4] and median transfusion requirement was 4 units [2–6]. In heart failure group, pretreatment median cardiac index was 4.5 L/min/m2 [4.1–5.6] and cardiac output was 8.3 L/min [7.5–9.2]. Bevacizumab 5 mg/kg/dose every 2 weeks for 6 applications was scheduled. By the end of induction, median hemoglobin at 3 months was 10.9 g/dl [9.5–12.8] (p = 0.01) and median transfusion requirement 0 units [0–1] (p<0.01), and this effect was more or less sustained during a year. Regarding heart failure group, two patients had complete hemodynamic response and achieved liver transplantation and two had partial response. No serious adverse events were registered.ConclusionBevacizumab is a promising line of treatment for HHT patients with refractory anemia. For patients with high output cardiac failure, bevacizumab may be useful as bridge therapy awaiting for liver transplantation.

Myocardite aiguë auto-immune secondaire à une maladie de Basedow : à propos d’un cas

IntroductionInteractions between heart and thyroid are strong. Main cardiac complications of Graves’ disease are supra-ventricular tachycardia or high output cardiac failure, without real myocardial involvement. ObservationA 40-year-old man with history of refractory Graves’ disease was hospitalized for an acute chest pain with elevated cardiac biomarkers and normal coronarography. Acute myocarditis was confirmed by cardiac MRI. We found no evidence for an infectious etiology. We retained the hypothesis of acute autoimmune myocarditis in the context of active Graves’ disease. ConclusionAcute myocarditis is an exceptional complication of Graves’ disease, with most likely an autoimmune mechanism. Possible occurrence of fulminant rhythmic or hemodynamic complications justify minimal cardiological check-up before introducing beta blockers.

Thyroid storm after coronary artery bypass surgery: a case report

BackgroundThyroid storm is a rare, life-threatening disease triggered by an acute event or trauma, such as surgery of the thyroid or another area, and infection. However, recent studies have shown that irregular use or discontinuation of antithyroid drugs is the most common cause of thyroid storm. A cardiovascular event caused by thyroid storm following coronary artery bypass graft (CABG) is high output heart failure with extreme tachycardia, which can be fatal. Thyroid storm after nonthyroidal surgery, especially CABG, has been rarely reported, with only one reported case until now. Herein, we present a case of thyroid storm onset in a patient who underwent CABG.Case presentationA 74-year-old woman with a history of antithyroid medication discontinuation against medical advice underwent urgent CABG. The patient exhibited extreme tachycardia postoperatively, which is highly suggestive of thyroid storm. Although a higher infection risk is an important consideration, a high-dose steroid was used to control the intractable tachycardia that did not respond to beta-blocker administration. Despite appropriate antibiotic treatment, the patient’s condition was exacerbated, and she developed multiple organ failure resulting from adult respiratory distress syndrome progression, and she died on day 8 after surgery.ConclusionsRisk factors for thyroid storm after CABG and its treatment outcomes are rarely reported. Patients with a history of inappropriate antithyroid medication prescription should be in a euthyroid state before surgery. If surgery is imminent, anticipating thyroid storm and its treatment as well as a euthyroid state can improve recovery outcomes postoperatively.

The Mechanism of High-Output Cardiac Hypertrophy Arising From Potassium Channel Gain-of-Function in Cantú Syndrome.

Dramatic cardiomegaly arising from gain-of-function (GoF) mutations in the ATP-sensitive potassium (KATP) channels genes, ABCC9 and KCNJ8, is a characteristic feature of Cantú syndrome (CS). How potassium channel over-activity results in cardiac hypertrophy, as well as the long-term consequences of cardiovascular remodeling in CS, is unknown. Using genome-edited mouse models of CS, we therefore sought to dissect the pathophysiological mechanisms linking KATP channel GoF to cardiac remodeling. We demonstrate that chronic reduction of systemic vascular resistance in CS is accompanied by elevated renin–angiotensin signaling, which drives cardiac enlargement and blood volume expansion. Cardiac enlargement in CS results in elevation of basal cardiac output, which is preserved in aging. However, the cardiac remodeling includes altered gene expression patterns that are associated with pathological hypertrophy and are accompanied by decreased exercise tolerance, suggestive of reduced cardiac reserve. Our results identify a high-output cardiac hypertrophy phenotype in CS which is etiologically and mechanistically distinct from other myocardial hypertrophies, and which exhibits key features of high-output heart failure (HOHF). We propose that CS is a genetically-defined HOHF disorder and that decreased vascular smooth muscle excitability is a novel mechanism for HOHF pathogenesis.