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1.
Fetal Diagn Ther ; 48(7): 560-566, 2021.
Artigo em Inglês | MEDLINE | ID: mdl-34412059

RESUMO

Placental chorangiomas can cause a high-output fetal state and increase neonatal morbidity and mortality. There is a paucity of data published describing the optimal treatment of these cases, and methods for occlusion to date include placement of vascular clips, bipolar cautery, injection of alcohol or surgical glue, interstitial laser, and microcoil embolization. We report 2 cases of prenatally diagnosed chorangiomas that caused a high-output fetal state and were successfully treated with microcoil embolization. This case series describes our technique and supports microcoil embolization as a potentially safe and effective antenatal treatment option in symptomatic chorangiomas.


Assuntos
Hemangioma , Doenças Placentárias , Feminino , Hemangioma/diagnóstico por imagem , Hemangioma/terapia , Humanos , Recém-Nascido , Placenta/diagnóstico por imagem , Doenças Placentárias/diagnóstico por imagem , Doenças Placentárias/terapia , Gravidez , Ultrassonografia , Ultrassonografia de Intervenção
2.
Am J Kidney Dis ; 71(6): 896-903, 2018 06.
Artigo em Inglês | MEDLINE | ID: mdl-29277506

RESUMO

Creation of an arteriovenous access for hemodialysis can provoke a sequence of events that significantly affects cardiovascular hemodynamics. We present a 78-year-old man with end-stage renal disease and concomitant coronary artery disease previously requiring coronary artery bypass grafting including a left internal mammary graft to the left anterior descending artery, ischemic cardiomyopathy with left ventricular systolic dysfunction, and severe aortic stenosis who developed hypotension unresponsive to medical therapy after recent angioplasty of his ipsilateral arteriovenous fistula for high-grade outflow stenosis. This case highlights the long-term effects of dialysis access on the cardiovascular system, with special emphasis on complications such as high-output cardiac failure and coronary artery steal syndrome. Banding of the arteriovenous fistula provided symptomatic relief with a decrease in cardiac output. Avoidance of arteriovenous access creation on the ipsilateral upper extremity in patients with a left internal mammary artery bypass graft may prevent coronary artery steal syndrome.


Assuntos
Doença da Artéria Coronariana/cirurgia , Reestenose Coronária/diagnóstico , Insuficiência Cardíaca/etiologia , Falência Renal Crônica/terapia , Diálise Renal/efeitos adversos , Dispositivos de Acesso Vascular/efeitos adversos , Idoso , Débito Cardíaco/fisiologia , Ponte de Artéria Coronária/métodos , Doença da Artéria Coronariana/complicações , Doença da Artéria Coronariana/diagnóstico por imagem , Reestenose Coronária/etiologia , Nefropatias Diabéticas/complicações , Nefropatias Diabéticas/diagnóstico , Progressão da Doença , Seguimentos , Insuficiência Cardíaca/diagnóstico , Insuficiência Cardíaca/terapia , Humanos , Falência Renal Crônica/diagnóstico , Masculino , Artéria Torácica Interna/transplante , Diálise Renal/métodos , Reoperação , Medição de Risco , Resultado do Tratamento
3.
Pediatr Radiol ; 48(8): 1172-1177, 2018 08.
Artigo em Inglês | MEDLINE | ID: mdl-29594439

RESUMO

Dysplastic megalencephaly, also known as bilateral hemimegalencephaly, is a rare cerebral malformation characterized by bilateral cerebral hemisphere overgrowth and extensive malformation of cortical development. Affected patients present clinically with intractable seizures, severe neurological impairment and global developmental delay. There is a small body of literature reporting megalencephaly's association with neonatal high-output cardiac failure and a lack of literature describing prenatal findings. We report a case of dysplastic megalencephaly presenting with progressive high-output cardiac failure during fetal life. Prenatal and postnatal imaging findings as well as neonatal course are described. A companion case with similar imaging findings will help illustrate the prenatal imaging characteristics of this association. Knowledge of this potential complication related to dysplastic megalencephaly may help guide parental counseling and obstetric management.


Assuntos
Insuficiência Cardíaca/congênito , Insuficiência Cardíaca/diagnóstico por imagem , Megalencefalia/diagnóstico por imagem , Adulto , Cesárea , Imagem de Difusão por Ressonância Magnética , Feminino , Humanos , Angiografia por Ressonância Magnética , Fenótipo , Gravidez , Ultrassonografia Pré-Natal
4.
J Korean Med Sci ; 31(12): 2051-2053, 2016 Dec.
Artigo em Inglês | MEDLINE | ID: mdl-27822948

RESUMO

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.


Assuntos
Fístula Arteriovenosa/complicações , Insuficiência Cardíaca/diagnóstico , Deslocamento do Disco Intervertebral/complicações , Abdome/diagnóstico por imagem , Adulto , Fístula Arteriovenosa/diagnóstico por imagem , Ecocardiografia , Procedimentos Endovasculares , Insuficiência Cardíaca/etiologia , Humanos , Hipertensão Pulmonar/diagnóstico por imagem , Artéria Ilíaca , Veia Ilíaca , Deslocamento do Disco Intervertebral/cirurgia , Vértebras Lombares/cirurgia , Masculino , Stents , Tomografia Computadorizada por Raios X , Veia Cava Inferior
5.
Pediatr Int ; 56(3): 304-8, 2014 Jun.
Artigo em Inglês | MEDLINE | ID: mdl-24689756

RESUMO

The International Society for the Study of Vascular Anomalies (ISSVA) classification divides vascular lesions into two major entities: neoplasms originating from the vascular endothelium and vascular malformations. Although this concept has been widely accepted, little has been established regarding vascular lesions in deep organs, such as infantile hepatic hemangioma (IHH). The current nationwide survey identified 19 critical infantile hemangiomas during the most recent 5 years. On histopathology all the lesions examined were neoplastic, but portovenos shunt was found histologically or clinically in some cases. High-output cardiac failure, consumption coagulopathy, and respiratory distress were the major symptoms, and treatment-resistant coagulopathy seemed to be the most reliable predictor of fatal outcome. Although steroid has been the gold standard treatment for these lesions, 25% of the patients were totally insensitive to steroids, whereas propranolol had a prompt effect in one case. For critical IHH with steroid-insensitive thrombocytopenia and prothrombin time prolongation, novel therapeutic options including beta-blocker therapy, surgery, and liver transplantation should be urgently considered as alterative treatment. The present review summarizes the results of the survey.


Assuntos
Hemangioma , Neoplasias Hepáticas , Coleta de Dados , Feminino , Hemangioma/epidemiologia , Hemangioma/terapia , Humanos , Lactente , Japão/epidemiologia , Neoplasias Hepáticas/epidemiologia , Neoplasias Hepáticas/terapia , Masculino
6.
ESC Heart Fail ; 11(1): 189-197, 2024 Feb.
Artigo em Inglês | MEDLINE | ID: mdl-37885349

RESUMO

AIMS: Patients with high-flow arteriovenous (AV) access are at risk of developing high-output cardiac failure (HOCF) and subsequent hospitalization. However, diagnosing HOCF is challenging and often requires invasive procedures. The role of systemic vascular resistance (SVR) in diagnosing HOCF is underestimated, and its predictive value is limited. Our study aims to identify non-invasive risk factors for HOCF to facilitate early diagnosis and timely surgical interventions. METHODS AND RESULTS: We included 109 patients with high-flow AV access who underwent serial echocardiography. The retrospective cohort was divided into two groups based on their hospitalization due to HOCF. The two groups were matched for age and gender. After a mean follow-up of 25.1 months, 19 patients (17.4%) were hospitalized due to HOCF. The two groups had similar baseline characteristics. However, the HOCF group had a higher value of vascular access blood flow (Qa) (2168 ± 856 vs. 1828 ± 617 mL/min; P = 0.045). Echocardiographic analysis revealed that the HOCF group had more pronounced left ventricular diastolic dysfunction (E/e': 21.1 ± 7.3 vs. 16.2 ± 5.9; P = 0.002), more severe pulmonary hypertension (right ventricular systolic pressure: 41.4 ± 16.7 vs. 32.2 ± 12.8; P = 0.009), a higher Doppler-derived cardiac index (CI) (4.3 ± 0.8 vs. 3.7 ± 1.1; P = 0.031), and a lower Doppler-derived estimated SVR (eSVR) value (5.5 ± 0.3 vs. 6.9 ± 0.2; P = 0.002) than the non-HOCF group. Using multivariable Cox regression analysis, a low eSVR value (<6) emerged as an independent predictor of HOCF hospitalization with a hazard ratio of 9.084 (95% confidence interval, 2.33-35.39; P = 0.001). Receiver operating characteristic curve analysis indicated that CI/eSVR values more accurately predicted HOCF hospitalization [sensitivity: 94.7%, specificity: 51.0%, area under the curve (AUC): 0.75, P < 0.001] than the Qa/cardiac output ratio (AUC: 0.50, P = 0.955), Qa values ≥ 2000 mL/min (AUC: 0.60, P = 0.181), and Qa values indexed for height in metres (AUC: 0.65, P = 0.040). CONCLUSIONS: In patients with high-flow AV access, low eSVR values obtained through non-invasive Doppler echocardiography were associated with a high rate of HOCF hospitalizations. Therefore, routine eSVR screening in these patients might expedite the diagnosis of HOCF.


Assuntos
Insuficiência Cardíaca , Humanos , Estudos Retrospectivos , Insuficiência Cardíaca/diagnóstico , Insuficiência Cardíaca/etiologia , Débito Cardíaco , Resistência Vascular , Ecocardiografia Doppler
7.
Cureus ; 16(6): e62780, 2024 Jun.
Artigo em Inglês | MEDLINE | ID: mdl-39036265

RESUMO

High-output cardiac failure is a less prevalent form of heart failure. Most patients with heart failure are typically categorized as having either systolic or diastolic dysfunction with elevated systemic vascular resistance. Individuals with high-output cardiac failure exhibit normal cardiac function and decreased systemic vascular resistance. This reduction may stem from diffuse arteriolar dilation or potential bypass of arterioles and capillary beds, prompting the activation of neurohormones. This case report details the diagnosis and treatment of an unusual etiology of high-output cardiac failure involving an arteriovenous fistula connecting the renal artery to the inferior vena cava and right common iliac vessels, resulting in a left-to-right shunt in a 50-year-old male patient. The report explores the etiology, pathophysiology, and clinical presentation of high-output heart failure, emphasizing the crucial role of radiology in interprofessional teams.

8.
Clin Transplant ; 27(6): 858-65, 2013.
Artigo em Inglês | MEDLINE | ID: mdl-24118251

RESUMO

BACKGROUND: Some hemodialysis patients develop arteriovenous (AV) fistulas with high flows. This volume overload can result in high-output cardiac failure. To date, predisposing access flow rates are unknown. METHODS: A retrospective study of all kidney-transplant recipients at the Medical University of Innsbruck (MUI) from 2005 to 2010 included 797 patients with the following criteria: previous hemodialysis with a native AV fistula or a graft, sufficient function of the kidney transplant up to the time of the data analysis, and follow-up care at the MUI. RESULTS: Twenty-nine of the 113 patients (25.7%) needed an AV fistula closure, mostly because of symptoms of cardiac failure. The mean shunt flow in the intervention group was 2197.2 mL/min, whereas the mean shunt flow in the non-intervention group was only 850.9 mL/min. Shunt closures were most frequently made in patients with upper-arm shunts (41.7%). CONCLUSION: The necessity of shunt closure is not a rarity. Patients who underwent an AV fistula ligature had high access flows with about 2200 mL/min. As the symptoms of cardiac failure greatly improved after shunt closure, patients with high access flow may benefit from such an intervention.


Assuntos
Fístula Arteriovenosa/etiologia , Insuficiência Cardíaca/etiologia , Falência Renal Crônica/cirurgia , Transplante de Rim , Complicações Pós-Operatórias , Diálise Renal/efeitos adversos , Áustria/epidemiologia , Feminino , Seguimentos , Insuficiência Cardíaca/diagnóstico , Humanos , Incidência , Masculino , Pessoa de Meia-Idade , Prognóstico , Estudos Retrospectivos , Fatores de Risco
9.
Cureus ; 15(7): e41942, 2023 Jul.
Artigo em Inglês | MEDLINE | ID: mdl-37588317

RESUMO

We present a case of a 67-year-old male with end-stage renal disease (ESRD) on hemodialysis who was admitted to the hospital after recurrent falls secondary to postural hypotension. He was not able to tolerate fluid removal on dialysis due to persistent hypotension despite maximal doses of midodrine and developed severe edema. A right heart catheterization revealed raised biventricular filling pressure consistent with right heart failure with low systemic vascular resistance and pulmonary hypertension. Duplex ultrasound of the left arm cephalic arteriovenous fistula (AVF) revealed a blood flow of 5.6 L/min. We hypothesized the cause of his high output heart failure from an AV fistula despite the lack of an increase in blood pressure after compression of the AVF. The AVF was ligated and a tunneled hemodialysis catheter was placed. Immediately after ligation, the patient was able to tolerate fluid removal with dialysis without hypotension, leading to a significant improvement in his edema and shortness of breath. This case highlights the potential adverse cardiovascular effects of AVF and the salutary effects on ligation in appropriate clinical settings.

10.
Front Med (Lausanne) ; 10: 1227283, 2023.
Artigo em Inglês | MEDLINE | ID: mdl-37928459

RESUMO

Most male X-linked Alport syndrome patients with COL4A5 nonsense mutations experience end-stage kidney failure by 30 years old. Although there is no definition of high-flow arteriovenous fistula, access blood flows greater than 2000 mL/min might predict the occurrence of high-output heart failure. A 50-year-old Japanese man had suffered from proteinuria at 4 years old and sensorineural hearing loss and a lenticular lens at 20 years old. He had started to receive hemodialysis treatment due to end-stage kidney disease at 22 years old. A genetic test confirmed a novel hemizygous nonsense variant COL4A5 c.2980G > T (p.Gly994Ter), and he was diagnosed with X-linked Alport syndrome. COL4A5 c.2980G > T was considered "pathogenic" according to the American College of Medical Genetics and Genomics guidelines and in vitro experiments. Shortness of breath on exertion was exaggerated, his brachial artery blood flow was over 4,236-4,353 mL/min, his cardiac output was 5,874 mL/min, and he needed radial artery banding at 51 years old. After radial artery banding surgery, the brachial artery blood flow decreased to 987-1,236 mL/min, and echocardiography showed a cardiac output at 5100 mL/min with improved E' and E/E'. His shortness of breath on exertion improved gradually. Although rare, high-output heart failure due to high-flow arteriovenous fistula should be kept in mind as a complication in X-linked Alport syndrome patients, and our patient was successfully treated with radial artery banding surgery.

11.
Front Cardiovasc Med ; 9: 949558, 2022.
Artigo em Inglês | MEDLINE | ID: mdl-36386341

RESUMO

Purpose: The aim of this study was to describe the treatment technique, outcomes, and complications of Schobinger stage IV head and neck arteriovenous malformations (HNAVMs) with associated high-output cardiac failure (HOCF) using ethanol and coils with the percutaneous suture technique. Methods: From January 2015 to December 2019, 19 patients who had HNAVMs with associated HOCF were treated first with a percutaneous suture of the remarkably dilated dominant drainage vein (RDDOV) and subsequent embolization with coils and ethanol. The percutaneous suture of RDDOV was preferred to be performed, followed by the deployment of coils and the injection of absolute ethanol via transarterial approach, direct puncture approach, or both of them. Treatment outcomes and complication rates were evaluated at follow-up. Results: A total of 19 patients who experienced HNAVMs with HOCF received 19 percutaneous suture procedures and 84 embolization procedures with ethanol and coils. Complete or >90% shunt reduction of the AVM was achieved in 16 patients. Notably, 19 patients with New York Heart Association (NYHA) stage II improved to stage I, and the symptom of dyspnea disappeared after embolization. The symptoms of five patients with bleeding disappeared. All patients presented with cosmetic concerns; Four were cured, and eight had a clearly recognizable improvement. Of note, 19 (100%) patients presented with impairment of daily life, which was resolved. The minor complications were encountered and recovered by the self. No major complications occurred. Conclusion: This study provides evidence that ethanol and coil embolotherapy is effective and safe in treating HOCF caused by HNAVMs with acceptable complications in these cases. The percutaneous suture technique for RDDOV management can act as an adjunct for embolotherapy.

12.
Curr Cardiol Rev ; 18(1): e050821195319, 2022.
Artigo em Inglês | MEDLINE | ID: mdl-34353268

RESUMO

INTRODUCTION: The knowledge on High-Output Cardiac Failure (HOCF) has greatly improved in the last two decades. One of the advances was the identification of a new phenotype of HOCF, characterized by the absence of ventricular dilation, already associated with liver disease, Arteriovenous Fistulas (AVF), lung disease, myelodysplastic syndromes, and obesity. However, it has been noted that any aetiology can present with one of the two phenotypes, depending on the evolution. OBJECTIVE: The study aims to describe, through an integrative review, the physiopathology and aetiologies of HOCF and to discuss phenotypes associated with this condition. METHODS: Revisions, guidelines, case-controls, cohort studies and clinical studies were searched in MEDLINE and LILACS, using the connectives in the "cardiac output, high" database (MeSH Terms) OR "high cardiac output" (All Fields). DISCUSSION: Two distinct phenotypes are currently described in the HOCF, regardless of the aetiology: 1) one with enlarged cardiac chambers; and 2) with normal heart chambers. The mechanisms related to HOCF are vasodilation, arteriovenous shunts that cause increased microvascular density, Reduced Systemic Vascular Resistance (RSVR), and high metabolism. These mechanisms lead to activation of the renin-angiotensin-aldosterone system, sodium and water retention, activation of neprilysin, of the sodium-glucose-2 transporter, which promote interstitial fibrosis, ventricular remodeling and a consequent increase in cardiac output >8L/min. CONCLUSION: Many aetiologies of HOCF have been described, and some of them are potentially curable. Prompt recognition of this condition and proper treatment may lead to better outcomes.


Assuntos
Insuficiência Cardíaca , Coração , Insuficiência Cardíaca/tratamento farmacológico , Humanos , Neprilisina/uso terapêutico , Fenótipo , Remodelação Ventricular
13.
Int J Angiol ; 31(2): 75-82, 2022 Jun.
Artigo em Inglês | MEDLINE | ID: mdl-35923582

RESUMO

The autosomal dominant trait hereditary hemorrhagic telangiectasia (HHT) causes multiorgan dysplastic lesions of the vasculature that can activate multiple physiological cascades leading to a broad array of cardiovascular diseases. Up to 78% of patients with HHT develop hepatic arteriovenous malformations (AVMs), which cause a hyperdynamic circulatory state secondary to hepatic/portal shunting. This condition can eventually progress to high-output cardiac failure (HOCF) with continued peripheral tissue hypoxemia. Treatment for HOCF is often limited to supportive measures (diuretics and treatment of anemia); however, recent studies using systemic bevacizumab have shown promise by substantially reducing the cardiac index. In the context of liver AVMs and high cardiac output, the pulmonary vasculature can also experience high flow. Without adequate dilation of pulmonary vessels, post-capillary pulmonary hypertension can develop. Another form of pulmonary hypertension observed in HHT, pulmonary arterial hypertension, is caused by HHT-related mutations in ENG and ACVRL1 causing congestive arteriopathy. Post-capillary pathogenesis is addressed by reducing the high-output state, whereas the pre-capillary state is treated with supportive mechanisms (diuretics, oxygen) and agents targeting pulmonary vasoreactivity: endothelin-1 receptor antagonists and phosphodiesterase-5 inhibitors. If either form of pulmonary hypertension is left untreated or proves refractory and progresses, the common hemodynamic complication is right heart failure. Targeted right heart therapies involve similar strategies to those of pulmonary arterial hypertension, with several experimental approaches under study. In this review, we describe in detail the mechanisms of pathogenesis, diagnosis, and treatment of the hemodynamic complications and associated cardiovascular diseases that may arise in patients with HHT.

14.
J Vasc Access ; 23(6): 864-870, 2022 Nov.
Artigo em Inglês | MEDLINE | ID: mdl-33971759

RESUMO

BACKGROUND: High-flow arteriovenous fistulae (HF-AVF) may lead to adverse cardiac remodeling in hemodialysis patients. We have investigated whether a novel external stent is safe and effective in reducing and stabilizing flow rates during a 1-year follow-up after HF-AVF reconstruction. METHODS: All patients with HF-AVF (access flow rate ⩾ 1500 ml/min), who underwent HF-AVF reconstruction with external stenting in two centers between June 2018 and May 2020, were included in this retrospective analysis. During HF-AVF reconstruction, the dilated vein segment was resected, underwent volume reduction, and was externally stabilized using a braided cobalt-chromium external stent. AVF flow rates were assessed preoperatively, intraoperatively, and at follow up visits using duplex ultrasound. RESULTS: Forty-three HF-AVFs in 42 patients were reconstructed and supported with an external stent (mean age 49 years, range 20-86 years; 74% men). Fifty-one percent were forearm AVFs, 49% were upper arm. The mean preoperative flow rate was 2622 ± 893 ml/min (range: 1500-6000 ml/min) and was decreased to 710 ± 221 ml/min (range: 300-1300 ml/min) intra-operatively after HF-AVF reconstruction. At 6 and 12-months follow-up, the mean flow rates were 1132 ± 320 ml/min (range: 470-1700 ml/min) and 1453 ± 888 ml/min (range: 300-3800 ml/min), respectively. Recurrence of high flow (>1500 ml/min) occurred in 16% and 25% of the patients at 6 and 12 months and primary patency rates were 86% and 70%, respectively. CONCLUSIONS: This early experience with novel external stenting for HF-AVF reconstruction demonstrates that it is a safe and effective method for reducing and stabilizing flow rates up to 1-year post procedure. Additional studies are required to evaluate the durability of this procedure over the longer term and assess its effect on cardiac remodeling.


Assuntos
Fístula Arteriovenosa , Derivação Arteriovenosa Cirúrgica , Masculino , Humanos , Adulto Jovem , Adulto , Pessoa de Meia-Idade , Idoso , Idoso de 80 Anos ou mais , Feminino , Derivação Arteriovenosa Cirúrgica/efeitos adversos , Derivação Arteriovenosa Cirúrgica/métodos , Grau de Desobstrução Vascular , Estudos Retrospectivos , Remodelação Ventricular , Resultado do Tratamento , Fatores de Tempo , Diálise Renal , Fístula Arteriovenosa/etiologia , Stents , Cromo , Cobalto
15.
J Am Heart Assoc ; 9(20): e016197, 2020 10 20.
Artigo em Inglês | MEDLINE | ID: mdl-33054561

RESUMO

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.


Assuntos
Débito Cardíaco Elevado , Estenose Subaórtica Fixa , Cardiopatias Congênitas , Insuficiência Cardíaca , Fígado , Telangiectasia Hemorrágica Hereditária , Receptores de Activinas Tipo II/genética , Débito Cardíaco Elevado/diagnóstico , Débito Cardíaco Elevado/etiologia , Débito Cardíaco Elevado/fisiopatologia , Estenose Subaórtica Fixa/diagnóstico , Estenose Subaórtica Fixa/genética , Estenose Subaórtica Fixa/fisiopatologia , Ecocardiografia/métodos , Feminino , Cardiopatias Congênitas/diagnóstico , Cardiopatias Congênitas/genética , Cardiopatias Congênitas/fisiopatologia , Insuficiência Cardíaca/diagnóstico , Insuficiência Cardíaca/etiologia , Insuficiência Cardíaca/fisiopatologia , Humanos , Fígado/irrigação sanguínea , Fígado/diagnóstico por imagem , Masculino , Pessoa de Meia-Idade , Mutação , Prognóstico , Estudos Retrospectivos , Análise de Sobrevida , Telangiectasia Hemorrágica Hereditária/diagnóstico , Telangiectasia Hemorrágica Hereditária/epidemiologia , Telangiectasia Hemorrágica Hereditária/genética , Telangiectasia Hemorrágica Hereditária/fisiopatologia , Estados Unidos/epidemiologia , Malformações Vasculares/diagnóstico , Malformações Vasculares/fisiopatologia
16.
Indian J Thorac Cardiovasc Surg ; 36(5): 515-517, 2020 Sep.
Artigo em Inglês | MEDLINE | ID: mdl-33061165

RESUMO

We report a case of a 24-year-old man who had an exploratory laparotomy 3 years earlier for a penetrating hypogastric wound. He presented with congestive heart failure that had progressed over the past 4 months. Transthoracic echocardiography and computed tomography (CT) angiography of the aorta demonstrated a wide arteriovenous fistula between the right common iliac artery and the iliocaval confluence, resulting in the dilatation of the inferior vena cava and of the right heart chambers. The patient was treated by surgical ligation of the right common iliac artery above and below the fistula in conjunction with aortoiliac bypass, thus excluding the shunt.

17.
Adv Chronic Kidney Dis ; 27(3): 214-218, 2020 05.
Artigo em Inglês | MEDLINE | ID: mdl-32891305

RESUMO

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 1.5 L 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.


Assuntos
Derivação Arteriovenosa Cirúrgica , Insuficiência Cardíaca , Falência Renal Crônica/terapia , Diálise Renal , Derivação Arteriovenosa Cirúrgica/efeitos adversos , Derivação Arteriovenosa Cirúrgica/métodos , Insuficiência Cardíaca/etiologia , Insuficiência Cardíaca/fisiopatologia , Insuficiência Cardíaca/prevenção & controle , Insuficiência Cardíaca/terapia , Humanos , Diálise Renal/efeitos adversos , Diálise Renal/métodos , Risco Ajustado/métodos , Volume Sistólico
18.
Mayo Clin Proc Innov Qual Outcomes ; 4(4): 460-463, 2020 Aug.
Artigo em Inglês | MEDLINE | ID: mdl-32793873

RESUMO

Bevacizumab is now an emerging treatment option for severe hereditary hemorrhagic telangiectasia-related bleeding including epistaxis and gastrointestinal tract bleeding. The impact of long-term intravenous bevacizumab therapy on cardiac structure and function is unknown. We describe 3 patients receiving intravenous bevacizumab therapy for severe hereditary hemorrhagic telangiectasia-related bleeding who were found to have abnormal mobile masses on the mitral valve (n=2) and aortic valve (n=1). The clinical impact of these findings is unknown and requires further study.

19.
Orphanet J Rare Dis ; 14(1): 256, 2019 11 14.
Artigo em Inglês | MEDLINE | ID: mdl-31727111

RESUMO

BACKGROUND: Systemic bevacizumab is a novel targeted anti-angiogenic therapy for high-output cardiac failure (HOCF) in hereditary hemorrhagic telangiectasia (HHT) but published data is limited. This survey-based study measured physician-reported safety, effectiveness and current treatment practices for systemic bevacizumab in HHT-HOCF. METHODS: A 27-item survey was sent to center directors of 31 international HHT Centers of Excellence. RESULTS: Response rate was 74% with centers reporting 150 total patients receiving systemic bevacizumab for HHT-HOCF. Approximately two-thirds of centers had treated ≥5 patients. All centers utilize a 5 mg/kg dose for induction treatment and most administer 6 doses (range, 4-6) every 2 weeks, although maintenance regimens varied considerably. Center directors reported bevacizumab to be effective, with 55% reporting significant improvement in cardiac index and HOCF symptoms in most patients treated with bevacizumab, although normalization of cardiac parameters was uncommon. Adverse events were uncommon with three-quarters of centers reporting adverse event rates < 10%. Discontinuation for adverse events or ineffectiveness was rare. Bevacizumab was typically administered by hematologists and pulmonologists (50 and 39% of centers, respectively), with highly variable thresholds for initiation. Although half the centers reported difficulty with the insurance approval process, 70% of centers were ultimately able to obtain coverage for most or all of their patients. CONCLUSIONS: Systemic bevacizumab is a widely-used therapy for HHT-HOCF with reasonable safety and effectiveness. HHT centers appear to vary considerably in maintenance treatment practices and disease severity thresholds for initiation of bevacizumab in HHT-related HOCF.


Assuntos
Antineoplásicos Imunológicos/uso terapêutico , Bevacizumab/uso terapêutico , Insuficiência Cardíaca/tratamento farmacológico , Insuficiência Cardíaca/etiologia , Telangiectasia Hemorrágica Hereditária/complicações , Telangiectasia Hemorrágica Hereditária/tratamento farmacológico , Adolescente , Adulto , Idoso , Feminino , Humanos , Masculino , Pessoa de Meia-Idade , Inquéritos e Questionários , Adulto Jovem
20.
J Nephrol ; 31(6): 975-983, 2018 Dec.
Artigo em Inglês | MEDLINE | ID: mdl-29357085

RESUMO

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.


Assuntos
Derivação Arteriovenosa Cirúrgica/efeitos adversos , Débito Cardíaco Elevado/diagnóstico por imagem , Ecocardiografia Doppler em Cores , Insuficiência Cardíaca/diagnóstico por imagem , Diálise Renal , Idoso , Velocidade do Fluxo Sanguíneo , Débito Cardíaco Elevado/etiologia , Débito Cardíaco Elevado/fisiopatologia , Estudos Transversais , Feminino , Insuficiência Cardíaca/etiologia , Insuficiência Cardíaca/fisiopatologia , Humanos , Masculino , Pessoa de Meia-Idade , Valor Preditivo dos Testes , Fluxo Sanguíneo Regional , Reprodutibilidade dos Testes , Medição de Risco , Fatores de Risco , Função Ventricular Esquerda
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