Severe heart failure, often with cardio-renal syndrome, is a despairing condition to treat. Rescue therapies such as LVAD are not widely available and cardiac transplantation is rarely used, even in developed countries. Subjects may be on optimum medical treatment and may have already been fitted with CRT-D in cases associated with LBBB.
Certain hidden aetiologies deserve mention.
Correcting iron deficiency, through IV iron infusion, can help.
Alcoholics deserve special mention. Heart failure in alcoholics is often confused with anasarca caused by cirrhosis. Many of these subjects have alcoholic cardiomyopathy, and in refractory, newly diagnosed heart failure, a history of alcohol abuse must always be sought.
Beri-beri is now considered very infrequently. First described in Japan in subjects consuming polished rice, cases seen in the West mainly comprise alcoholics. However, severe thiamine deficiency occurs quickly in subjects with persistent vomiting, after bariatric surgery, after parenteral nutrition, and in cancer patients or other subjects with poor nutrition. Refeeding increases thiamine requirements, as does dextrose infusion.
Dry beri-beri is due to peripheral neuropathy, usually presenting with burning pain the feet and a glove and stocking distribution of patchy sensory loss. Wet beri-beri in its classic form is considered to be a phenotype of hyperdynamic, mainly right sided failure associated with peripheral vasodilatation. The left heart is largely spared.
However, there is another form of beriberi, called "Shoshin beriberi" which may not be widely known. ("Sho" means acute in Japanese, while "shin" denotes heart). This can cause acute bi-ventricular heart failure and if untreated, can be fatal within hours in advanced cases. In the two cases described by Wolf and Levin in The New England Journal of Medicine in 1960, subjects had striking peripheral cyanosis affecting the extremeties, tachycardia, cardiomegaly, hypotension, and pulmonary oedema. Both were alcoholics and one hadn't eaten for six days, subsisting on wine. There was striking improvement with IV thiamine in one patient, while the other case was only diagnosed at autopsy.
Another under-appreciated sign of thiamine deficiency in such subjects is lactic acidosis. This acidosis often drives severe tachypnoea and thus such subjects may have a paradoxically normal oxygen saturation, despite striking peripheral cyanosis. Thus, an alcoholic or otherwise malnourished subject presenting in acute pulmonary oedema, associated with anxiety, restlessness, dyspnoea and often central chest discomfort, with lactic acidosis on venous blood gas often upto 6-7 mmol/l, should be considered to have Shoshin beriberi. Intravenous thiamine may be lifesaving in such cases, and should be administered without delay even if a history of alcohol abuse is not immediately forthcoming. Whole blood thiamine or erythrocyte transketolase levels should be sent off concurrently.
It is well recognised that loop diuretics cause increased loss of thiamine in the urine. However, there is disagreement on whether this has the potential to worsen heart failure.
For some reason, Wernicke's encephalopathy and beri-beri do not co-exist very often. These disparate signs of severe thiamine deficiency are therefore rarely seen together.
Certain hidden aetiologies deserve mention.
Correcting iron deficiency, through IV iron infusion, can help.
Alcoholics deserve special mention. Heart failure in alcoholics is often confused with anasarca caused by cirrhosis. Many of these subjects have alcoholic cardiomyopathy, and in refractory, newly diagnosed heart failure, a history of alcohol abuse must always be sought.
Beri-beri is now considered very infrequently. First described in Japan in subjects consuming polished rice, cases seen in the West mainly comprise alcoholics. However, severe thiamine deficiency occurs quickly in subjects with persistent vomiting, after bariatric surgery, after parenteral nutrition, and in cancer patients or other subjects with poor nutrition. Refeeding increases thiamine requirements, as does dextrose infusion.
Dry beri-beri is due to peripheral neuropathy, usually presenting with burning pain the feet and a glove and stocking distribution of patchy sensory loss. Wet beri-beri in its classic form is considered to be a phenotype of hyperdynamic, mainly right sided failure associated with peripheral vasodilatation. The left heart is largely spared.
However, there is another form of beriberi, called "Shoshin beriberi" which may not be widely known. ("Sho" means acute in Japanese, while "shin" denotes heart). This can cause acute bi-ventricular heart failure and if untreated, can be fatal within hours in advanced cases. In the two cases described by Wolf and Levin in The New England Journal of Medicine in 1960, subjects had striking peripheral cyanosis affecting the extremeties, tachycardia, cardiomegaly, hypotension, and pulmonary oedema. Both were alcoholics and one hadn't eaten for six days, subsisting on wine. There was striking improvement with IV thiamine in one patient, while the other case was only diagnosed at autopsy.
Another under-appreciated sign of thiamine deficiency in such subjects is lactic acidosis. This acidosis often drives severe tachypnoea and thus such subjects may have a paradoxically normal oxygen saturation, despite striking peripheral cyanosis. Thus, an alcoholic or otherwise malnourished subject presenting in acute pulmonary oedema, associated with anxiety, restlessness, dyspnoea and often central chest discomfort, with lactic acidosis on venous blood gas often upto 6-7 mmol/l, should be considered to have Shoshin beriberi. Intravenous thiamine may be lifesaving in such cases, and should be administered without delay even if a history of alcohol abuse is not immediately forthcoming. Whole blood thiamine or erythrocyte transketolase levels should be sent off concurrently.
It is well recognised that loop diuretics cause increased loss of thiamine in the urine. However, there is disagreement on whether this has the potential to worsen heart failure.
For some reason, Wernicke's encephalopathy and beri-beri do not co-exist very often. These disparate signs of severe thiamine deficiency are therefore rarely seen together.
