TITLE

Non compaction cardiomyopathy and idiopathic retroperitoneal fibrosis: A rare occurrence

INTRODUCTION
Isolated Non compaction of the left entricle !L"NC# is a rare disorder$ classified as primary
%enetic cardiomyopathy by the American heart Association& L"NC 'as preiously named
spon%y myocardium or hypertrabeculation syndrome& The prealence of L"NC is not (no'n but
a reie' for )'it*erland identified +, cases 'ithin -. years$ 'hich represented /&/-,0 of
echocardio%rams that 'ere performed& 1o'eer$ this probably mi%ht be an underestimate$
since increased echocardio%raphic ima%e 2uality and increasin% a'areness of L"NC 'ill li(ely
lead to enhanced reco%nition&
Retroperitoneal fibrosis is a rare entity characterised by inflammation and fibrosis of
retroperitoneal tissue that often encases the ureters and abdominal or%ans&
3e report a case of refractory con%estie heart failure in a patient 'ith L"NC complicated by
pro%ressie renal failure due to retroperitoneal fibrosis&
CA)E 4RE)ENTATION
,5 year old African American male 'ith chronic (idney disease secondary to retroperitoneal
fibrosis presented to the hospital 'ith complaints of pro%ressie dyspnea$ fati%ue and le%
s'ellin%& 4hysical e6am 'as si%nificant for eleated 7u%ular enous pressure$ bibasilar crac(les$
pansystolic murmur at the ape6 radiatin% to the a6illa and pittin% edema& Echocardio%ram
sho'ed prominent trabeculation inn the ape6 consistent 'ith L"NC 'ith seere L" systolic
dysfunction$ E8 -/9-.0 and moderately reduced R" systolic function&
The patient 'as admitted to the cardiac ICU 'here he 'as started on diuretics$ beta bloc(ers
and also re2uired intraenous inotropes& 1is hospital course 'as complicated by 'orsenin%
renal failure re2uirin% intermittent hemodialysis and an acute I"C thrombus&
DI)CU))ION
This case is remar(able due to the occurrence of t'o rare clinical entities in a sin%le patient&
The patient:s underlyin% chronic (idney disease 'orsened 'ith acute decompensated heart
failure& Retroperitoneal fibrosis often presents 'ith systemic hypertension$ left entricular
hypertrophy and deep enous thrombosis& In a population based study in Netherlands$ the
reported annual incidence 'as appro6imately -&+ per -//$/// inhabitants per year&
L"NC is a rare con%enital cardiomyopathy that appears to result from intrauterine arrest of
endomyocardial morpho%enesis&;-< T'o forms hae been described: an isolated form and a
non isolated form associated 'ith other con%enital cardiac defects& In the latter form entricular
septal defect$ pulmonic stenosis$ hypoplastic left entricle hae been described to coe6ist& The
classic clinical manifestations include heart failure$ arrhythmias and thromboembolic eents&
Treatment includes follo'in% the standard %uidelines for heart failure$ AICD for primary
preention of sudden cardiac death and eentual heart transplant&
CONCLU)ION
Left entricular non9compaction !L"NC# is a rare sporadic or familial cardiomyopathy& The
clinical presentations and pro%nosis of such patients are ariable= includin% heart failure$ chest
pain$ thromboembolic eents and atrial and entriculararrhythmias& L"NC patients 'ho hae
end9sta%e heart failure are
candidates for cardiac transplantation ealuation&
RE8ERENCE)