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.

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.

Estimated cardiac output and cardiovascular profile score in fetuses with high cardiac output lesions.

OBJECTIVE: High cardiac output lesions are associated with an increased risk of fetal death, largely as a result of cardiac failure and hydrops fetalis. The cardiovascular profile score (CVPS) has been used to characterize cardiovascular wellbeing, and has been linked to fetal outcomes in other conditions. We aimed to test the hypothesis that elevated combined cardiac output (CCO) in fetuses with high output lesions may be associated with worsening cardiovascular status, as evidenced by a lower CVPS. METHODS: A retrospective review was performed of fetuses with high cardiac output lesions that underwent echocardiography between July 2006 and November 2010. Diagnoses included sacrococcygeal teratoma, placental chorioangioma and vein of Galen aneurysm. Fetal echocardiographic evaluation included assessment of CVPS, as well as Doppler/two-dimensional estimation of CCO, indexed to estimated fetal weight (CCOi). The relationship between CCO and CVPS was assessed. RESULTS: A total of 35 fetuses were studied: 27 had sacrococcygeal teratoma, seven had chorioangioma and one had vein of Galen aneurysm. There was a significant inverse relationship between mean logCCOi and CVPS (r2 = 0.48, P = 0.008). Of 31 patients with clinical outcome data, 10 experienced either in-utero demise or intervention; 80% of these fetuses had a CVPS of < 8. CONCLUSIONS: There is an inverse relationship between CCO and CVPS in the fetus with high cardiac output lesions. As a measure of fetal cardiovascular wellbeing in this population, the CVPS may be a useful tool for stratifying risk and for selection for intervention in these fetuses.

Eight-year follow-up after palliative embolization of a neonatal intracranial dural arteriovenous fistula with high-output heart failure: management strategies for symptomatic fistula growth and bilateral femoral occlusions in pediatric patients.

The authors report the 8-year follow-up of a patient previously described in the literature who originally presented in high-output cardiac failure secondary to a complex neonatal intracranial dural arteriovenous fistula (DAVF). The earlier case report described palliative treatment with a combination of extracorporeal membrane oxygenation (ECMO) and endovascular embolization for life-threatening high-output cardiac failure secondary to a DAVF. Access was obtained using the ECMO cannula, and embolization was performed while the patient was connected to the ECMO machine. The patient made an excellent recovery following partial embolization of the fistula, but then presented again 7 years later with worsening headaches secondary to significant growth of the known residual portion of the fistula identified on CT angiography. The child also developed bilateral femoral artery (FA) occlusions secondary to multiple previous FA punctures. To achieve complete obliteration of the remaining fistula, the patient required a retroperitoneal approach to the iliac artery and percutaneous puncture of the internal jugular vein. Embolization was performed with a combination of platinum coils and ethylene vinyl alcohol copolymer liquid embolic agent. There were no complications, and the child remains neurologically normal, with no signs of permanent cardiovascular sequelae. In this case report, the authors discuss the long-term management of AVFs treated by endovascular strategies early in life. After neonatal access, sometimes the FAs occlude, requiring more invasive access strategies. The authors also discuss the follow-up method, intervals, and threshold for further treatment for these lesions, and present a review of the literature.

High output heart failure.

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

Mandibular gingival arteriovenous malformation in pregnancy.

Arteriovenous malformations (AVM) are rare in the oral and maxillofacial regions. We report a case of a mandibular gingival AVM in a pregnant woman, who presented at 35 weeks' gestation with massive pulsatile bleeding from a mass in the mandibular gingival margin, which could be managed by local compression and cauterisation. After delivery, the mass gradually regressed. She developed a similar swelling during a second pregnancy. It was thought that increased cardiac output as a result of her pregnancy caused her AVM to progress.

Sudden death after arteriovenous fistula ligation in a renal transplant patient.

After a period of time using an arteriovenous (AV) fistula as vascular access for hemodialysis, kidney transplant recipients usually undertake surgical closure if the fistula is not spontaneously closed. In all prospective studies addressing this issue, absence of major adverse events and progressive decrease in left ventricular volume and mass is the rule. However, in these studies, patients with heart failure New York Heart Association (NYHA) III or IV were not included, and consequently, the effects of AV ligation in these high-risk patients are not well known. We present a heart failure NYHA IV renal transplant patient with fatal evolution after surgical closure of her high-flow AV fistula.

Acute volume overload elevates T-wave alternans magnitude.

The objective of this study was to determine whether acute volume loading elevates T-wave alternans (TWA) in dogs with structurally normal hearts. TWA predicts sudden cardiac arrest in patients with left ventricular dysfunction and congestive heart failure. However, volume load and ventricular stretch may themselves precipitate arrhythmias. It is unclear to what extent volume load causes TWA. In six male mongrel dogs [25.8 kg (SD 4.2)] under general anesthesia, we measured TWA during progressive atrial pacing to 160 beats/min. Pacing was performed at baseline, at the midpoint and peak of a saline infusion designed to induce acute CHF, and then during diuresis. Dog 1 was hypothermic throughout the protocol and excluded from analysis. For dogs 2-6, 102 ml/kg (SD 30) were infused over 315 min (SD 50), causing pulmonary capillary wedge pressure to rise from 9.6 (SD 3.5) to 21.2 mmHg (SD 1.6) (P < 0.01), and heart rate variability to fall (P < 0.01). TWA magnitude (Valt) rose in all dogs with volume load (P < 0.001). Compared with baseline, TWA at peak infusion had higher magnitude [Valt 3.4 (SD 1.95) vs. 0.5 muV (SD 0.35); P = 0.011] and occurred at lower heart rates [128 (SD 6) vs. 151 beats/min (SD 12); P = 0.008]. Net volume load was linearly related to Valt (P < 0.01), with each 10 ml/kg net volume load increasing Valt by 0.23 muV. Acute volume overload elevates TWA in normal canine hearts. Although dramatic, however, this effect may contribute clinically to abnormal TWA only in patients with marked volume overload. Future studies should examine the interaction of fluid overload, myocardial disease, and arrhythmia susceptibility.

Successful anesthetic management of a child with an extensive facial hemangioma and high output cardiac failure for placement of a central venous catheter.

A two-and-a-half-year-old female with an extensive facial and lingual hemangioma, associated high output cardiac failure, and a history of difficult intubation presented for central venous catheter (CVC) placement for vincristine chemotherapy. Anesthetic management of this complex case is presented and the complicating medical conditions are discussed.

[Highly concentrated vascular endothelial growth factor in Crow-Fukase syndrome with high output heart failure: a case report].

Crow-Fukase syndrome is a disease of plasma cell dyscrasia. Congestive heart failure is the biggest complication affecting the prognosis. A 57-year-old male was admitted with edema and low grade fever. Globe and stocking type polyneuropathy, increased levels of adrenocorticotropic hormone and thyroid-stimulating hormone, serum M-protein component of the immunoglobulin A-lambda type, skin polypoid lesion, and organomegaly including cardiomegaly were observed. The diagnosis was Crow-Fukase syndrome based on these clinical features. High output heart failure and pulmonary hypertension were determined with a cardiac catheter. Diuretics and angiotensin converting enzyme inhibitor were effective to control his overhydration. The level of serum vascular endothelial growth factor was markedly increased and might be responsible for the manifestation of this syndrome with cardiac involvement.

Hepatic artery embolization for treatment of patients with hereditary hemorrhagic telangiectasia and symptomatic hepatic vascular malformations.

At present there is no established therapy for treating patients with hereditary hemorrhagic telangiectasia (HHT) and symptomatic hepatic involvement. We present the results of a prospective study with 15 consecutive patients who were treated with staged hepatic artery embolization (HAE). Branches of the hepatic artery were selectively catheterized and embolized in stages using polyvinyl alcohol particles (PVA) and platinum microcoils or steel macrocoils. Prophylactic antibiotics, analgesics and anti-emetics were administered after every embolization. Clinical symptomatology and cardiac output were assessed before and after therapy as well as at the end of follow-up (median 28 months; range 10-136 months). Five patients had abdominal pain and four patients had symptoms of portal hypertension. The cardiac output was raised in all patients, with cardiac failure being present in 11 patients. After treatment, pain resolved in all five patients, and portal hypertension improved in two of the four patients. The mean cardiac output decreased significantly ( P<0.001) from 12.57+/-3.27 l/min pre-treatment to 8.36+/-2.60 l/min at the end of follow-up. Symptoms arising from cardiac failure resolved or improved markedly in all but one patient. Cholangitis and/or cholecystitis occurred in three patients of whom two required a cholecystectomy. One patient with pre-existent hepatic cirrhosis died as a complication of the procedure. Staged HAE yields long-term relief of clinical symptoms in patients with HHT and hepatic involvement. Patients with pre-existing hepatic cirrhosis may be poor candidates for HAE.

Arteriovenous fistula-associated high-output cardiac failure: a review of mechanisms.

High-output cardiac failure can be a rare complication of high-output arteriovenous fistula. The authors present a case in which a hemodialysis patient with a high-flow arteriovenous fistula has cardiac failure that improves with fistula closure. The hemodynamic effects of a fistula are reviewed, and the hemodialysis literature regarding high-output cardiac failure is summarized. To gain insight into the problem of high-output cardiac failure, research efforts should focus on the prospective monitoring of high-access flows.

Cardiac abnormalities associated with hydrops fetalis.

Cardiovascular abnormalities are associated with hydrops fetalis in 26% of cases reported in the modern era. These include not only structural abnormalities, but also cardiac arrhythmias, failure, tumors, myopathy, infection, inflammation, infarction, and arterial calcification. Cardiac structural abnormalities may be causative or seen only in association with hydrops fetalis. Structural lesions that result in right atrial pressure or volume overload seem to be most commonly associated with hydrops fetalis. Fetal cardiac tumors, cardiomyopathy, myocarditis, myocardial infarction, and arterial calcification probably result in hydrops fetalis by a similar mechanism. Fetal tachyarrhythmia has been shown to result in elevation of atrial pressure and atrial natriuretic peptide. Fetal tachyarrhythmias are the most treatable of cardiac causes of hydrops fetalis. Fetal bradyarrhythmias are less easily treatable and less certainly a causative mechanism of hydrops fetalis.

[Hypercirculatory heart failure in a patient with plasmacytic leukemia]. / Hypercirkulacné zlyhanie srdca u chorého s plazmocytovou leukémiou.

High cardiac output failure/state (HCOF) is regular feature of some illnesses e.g. thiamine deficiency, hyperthyroidism, severe anemia, Paget's disease or arteriovenous fistulae. HCOF in multiple myeloma is reported quite rarely. 31-year-old man was admitted because of fatigue, dyspnea and subfebrilities. Heart rate was 116/min, sinus rythm blood pressure 110/60 mmHg. Chest film showed cardiomegaly with sings of interstitial pulmonary edema, echocardiography mild dilatation of the left ventricle with hyperkinetic wall motion and small pericardial effusion. Hemoglobin was 104 g/l, leukocyte count 13.5 x 10(9)/l with 30% of plasmatic cells. Serum protein electrophoresis demonstrated a monoclonal gammapathy, X ray studies of the skelet multiple osteolytic lesions. Diagnosis of plasmocytic leukemia-form of multiple myeloma was established and chemotherapy (vincristine + adriamycine + dexamethason) was started. Patient cardiac status deteriorated. Cardiac catheterisation demonstrated mean righ atrial pressure of 25 mmHg, mean pulmonary artery pressure of 28 mmHg and pulmonary artery wedge pressure of 24 mmHg. Co was 20.0 l/min (C.I. 11.5 l/min/m2). In continuing of chemotherapy and symptomatic therapy for heart failure patients status gradually improved and complete remission of the myeloma and normalisation of cardiac parameters was achieved. Heart failure in multiple myeloma patients has been attributed to amyloidosis of myocardium, hyperviscosity syndrome, co-existing CAD or anthracycline toxicity. HCOF should be considered in patients with clinical evidence of heart failure and normal left ventricular function.