Hjärtsvikt i samband med HHT
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Two Cases of High Output Heart Failure Caused by Hereditary Hemorrhagic Telangiectasia
"High-output cardiac failure is a rare complication of hereditary hemorrhagic telangiectasia (HHT) usually caused by shunting of blood through atriovenous malformations (AVMs) in the liver. We describe two cases of high output heart failure due to large hepatic AVMs. Clinical suspicion of HHT based on detailed history taking and physical examination is essential for early detection and proper management of heart failure associated with HHT."
"High-output cardiac failure is a rare complication of HHT usually caused by shunting of blood through AVMs in the liver. We describe two cases of high output heart failure due to large hepatic AVMs, highlighting their importance in the differential diagnosis of heart failure syndromes."
"Hereditary hemorrhagic telangiectasia or Osler-Weber-Rendu disease, characterized by telangiectases and AVM in multiple organs is a rare disease with autosomal dominant transmission. The classic triad is recurrent epistaxis, mucocutaneous telangiectasia, and familial occurrence. The diagnosis of HHT is confirmed if any three of the following four criteria are met: 1) recurrent epistaxis; 2) multiple telangiectasias; 3) presence of visceral lesions (in the gastrointestinal tract, lung, liver, brain); 4) first degree family history of HHT."
"Hepatic involvement in HHT has been reported in 74% of patients with CT of the abdomen. But liver involvement tends to be asymptomatic in majority of cases and the frequency of symptomatic hepatic involvement is less than 10%. Although heart failure secondary to the hyperdynamic circulatory situation caused by arteriovenous fistulas is a well-known clinical entity, the clinical presentation of heart failure is very rare in HHT."
"The shunting of blood through the AVMs, especially in the liver leads to maldistribution of cardiac output. In order to supply blood to vital organs, cardiac output is increased with elevated stroke volume and heart rate, leading to high output heart failure. A high cardiac output has been described as being >8 L/min or a cardiac index >3.9 L/min/m2. Right heart catheterization in our case 1 demonstrated a markedly increased cardiac output of 12.47 L/min. Reported cases of right-heart catheterization in patients with symptomatic hepatic AVMs have calculated shunts of 24% to 58% of the cardiac output. The echocardiography usually shows compensatory left ventricular dilatation and left ventricular hypertrophy, but preserved or high left ventricular systolic function. High output heart failure could be presented as a diastolic dysfunction. Echocardiographic findings in our cases were consistent with those of the previous reports."
"In contrast to low output heart failure, clinical trial data regarding treatment of high output heart failure are lacking. The treatment of high output heart failure in patients with HHT is usually conservative. Diuretics are the mainstay of treatment in high output heart failure, especially with symptoms of volume overload. The patients in the two above mentioned cases were treated with angiotensin receptor blocker and diuretics. The treatment was effective for managing their symptoms related to heart failure. This treatment may not be effective in patients with large vascular malformations. There have been scattered experiences with transfemoral embolization, surgical ligation of the feeding vessels in symptomatic patients. However, in intractable patients, liver transplantation has been the standard treatment. Recently, treatment with bevacizumab-the anti-vascular endothelial growth factor has been reported to be effective in heart failure patients with HHT by slowing the progression of vascular malformations."
"In conclusion, we present here two patients of HHT who presented with high output heart failure. High output heart failure was controlled by medical management with some clinical improvement, but a careful follow-up may be necessary to detect developing cerebral or pulmonary AVMs. Clinical suspicion of HHT based on detailed history taking and physical examination is essential for early detection and proper management of heart failure associated with HHT."
Källa: Korean Circ J. 2012 Dec;42(12):861-865.Published online December 31, 2012. doi: https://dx.doi.org/10.4070%2Fkcj.2012.42.12.861