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despite recent advances in cardiopulmonary support strategies , dilated cardiomyopathy ( dcmp ) remains a life threatening disease in young patients.1)2 ) early recognition and treatment of dcmp are crucial for good prognoses in this patient population.3)4 ) the clinical course of patients with dcmp that result in cardiogenic shock varies according to the etiology of their disease as well as patient age .
several cases of dcmp complicated by sleep apnea syndrome have reportedly been managed with continuous positive airway pressure.5 ) the volumetric expansion of the enlarged heart can compress adjacent structures in the thoracic cavity causing a number of related symptoms , especially in infants with soft cartilaginous bronchi.6)7 ) therapeutic strategies for treating these issues vary according to the type of complication encountered .
a corrected age 2-month - old female infant presented to our hospital with poor feeding and irritability for 7 days .
the infant was delivered prematurely at our hospital with a birth weight of 2.45 kg ( 50 - 75 percentile ) and a height 47 cm ( 75 percentile ) at 34 weeks of gestation ( fig .
the patient 's body weight on admission was 5.1 kg ( 10 - 25 percentile ) and height 60 cm ( 75 - 90 percentile ) .
the patient exhibited signs and symptoms of shock with blood pressure 54 mmhg ( in systole ) and 31 mmhg ( in diastole ) , heart rates 200 beats per minute , respiration rates 60 per minutes , body temperature 36.6. chest roentgenogram showed unilateral haziness of the entire left lung and suspected cardiomegaly , while the right lung field appeared to be normal ( fig .
echocardiography revealed a massively dilated left ventricle ( lv ) with poor contractility ( fig .
there was also evidence of severe atrioventricular valve regurgitation ( fig . 2 b ) , and the lv chamber appeared spherical in shape
the patient 's lv ejection fraction ( ef ) was estimated to be 24.5% , with a diastolic lv inner diameter of 41.3 mm , end point septal separation of the anterior mitral valve of 17.3 mm ( fig . 2 d ) , and spherical index of 0.92 ( reference ; 0.60 - 0.73 ) ( table 1 ) .
in contrast to the markedly elevated n - terminal pro brain natriuretic peptide of > 35000 pg / ml and severe anemia with a hemoglobin of 6.5 g / dl , other cardiac enzymes including serum glutamic oxaloacetic transaminase , creatine phosphokinase , lactate dehydrogenase , creatine phosphokinase - mb , troponin - t , and c - reactive protein remained within normal limits throughout the patient 's hospital stay ( table 2 ) .
1 a ) , but no abnormal cardiopulmonary signs or symptoms were noted with respect to the previously recorded vital signs . with aggressive cardiopulmonary support including inotropes ( dopamine , dobutamine , and milrinone ) , diuretics , and continuous nitroglycerine ( 0.5 g / kg / min ) infusion with shock management , vital signs were relatively stable by the 3rd day of admission .
extracorporeal membrane oxygenator support or ventilator care were not applied throughout the entire course in intensive care unit .
studies performed to identify the etiology of dcmp including tests for enterovirus , adenovirus , cytomegalovirus , rubella , measles , epstein - barr virus , mumps , parvovirus , herpes virus , varicella - zoster , the hepatitis viruses , and cultures for several kinds of bacteria .
these studies along with measured carnitine levels yielded non - specific findings . while lv systolic function improved gradually on echocardiography , the spherical contour and markedly increased inner diameter of the lv and valvular insufficiency showed little interval change prior to discharge .
intravenous medications such as inotropes and nitroglycerine were switched to oral medications including an angiotensin converting enzyme inhibitor , digoxin , and diuretics 10 days after admission as the patient was transferred to the general ward . on the 24th day of admission ,
the patient was discharged home in stable condition with a lvef of 35%(table 1 ) .
two months later at an outpatient follow - up visit , the patient continued to have decreased breath sounds with total haziness of the left lung field on roentgenography , making it difficult to assess the cardiac silhouette and size ( fig .
chest computed tomography ( ct ) ( fig . 3a , b , and c ) with 3 dimensional ( 3d ) reconstruction of the airway revealed severe external compression of the left main stem bronchus between the enlarged left atrium and the aorta , resulting in atelectasis in the left lung ( fig .
as the patient did not show any signs of respiratory distress , she was treated with conservative management .
the patient showed normal growth and development without any neurologic complications . with gradual improvement of cardiomegaly and lv function ,
the atelectasis resolved spontaneously within 2 months ( fig . 1 d and e ) .
after a year of treatment with oral carvedilol , follow - up echocardiography showed normal lv systolic function and nearly normal lv contour ( fig .
one year from the onset of symptoms and signs , as an 14 month old female infants , the patient showed normal growth with body weight 11.4 kg ( 25 - 50 percentile ) , height 77 cm ( 25 - 50 percentile ) and normal development without any neurologic complications .
it is often difficult to differentiate between dcmp and myocarditis . since the patient in this case experienced sudden onset of massive cardiomegaly with fulminant heart failure
dcmp typically causes dilatation of the mitral valve annulus , resulting in valve insufficiency and left atrial enlargement . due to
the intimate relationship between the airway and the left atrium , left pulmonary veins , and left pulmonary artery , if the mean pulmonary arterial pressure , mean left atrial pressure , and carinal angle increase , the likelihood of major airway compression is high.8 ) however , in this case the left atrium was not severely enlarged despite the nearly perfect spherical dilation of the lv chamber . in very young infants , soft cartilaginous main stem bronchi can be easily externally compressed .
this results in atelectasis that is difficult to manage , and surgical intervention is sometimes required.9 ) in this case , the obstruction of the left main bronchus resolved completely without surgical intervention after 3 months of prolonged compression by the enlarged heart .
considering the various geometric changes that can occur with cardiomegaly , interventional strategies could potentially be tailored on a case by case basis with the help of cardiac ct imaging.10 ) we report a case of severe dcmp with massive cardiomegaly complicated by bronchial obstruction in which 3d ct was helpful for assessing detailed airway patency and choosing therapeutic strategies in an infant .
dilated cardiomyopathy ( dcmp ) remains a life threatening disease in young patients and is often difficult to differentiate from myocarditis . 
 early recognition and treatment of dcmp are crucial for good prognoses in this patient population . 
 the clinical course of patients with dcmp that result in cardiogenic shock varies according to the etiology as well as patient age . 
 the volumetric expansion of the enlarged heart can compress adjacent structures causing a number of related symptoms , especially in infants with soft cartilaginous bronchi . 
 therapeutic strategies for treating these issues vary according to the type of complication encountered . 
 we report a case of severe dcmp with sudden onset of massive cardiomegaly with heart failure complicated by bronchial obstruction in an infant .