Left ventricular noncompaction (LVNC) is a rare cardiomyopathy characterized by a hypertrabeculation of the left ventricle. Patients may present with heart failure, arrhythmia, and thromboembolism. LVNC may be isolated or associated with congenital heart defects. The first discovered genetic cause of isolated LVNC was Barth syndrome (BTHS), an X-linked disorder caused by taffazin (TAZ) gene mutation. BTHS is characterized by cardiomyopathy, neutropenia, skeletal myopathy, and growth delay. A newborn male baby was referred to Soonchunhyang University Cheonan Hospital due to cyanosis and dyspnea. Based on findings of cardiomegaly, ST depression, and elevated cardiac enzyme, echocardiography was done, which revealed a hypocontractile, enlarged left ventricle with distinctive trabeculation in the apex. Heparinization for the treatment of myocardial infarction and continuous infusion of milrinone was started. During hospitalization, the TAZ gene mutation was detected in the patient, his mother, and elder sister. After 3 months, the patient was discharged with heart failure medication and aspirin.
Arrhythmias, Cardiac ; Aspirin ; Barth Syndrome* ; Cardiomegaly ; Cardiomyopathies ; Chungcheongnam-do ; Cyanosis ; Depression ; Dyspnea ; Echocardiography ; Heart Defects, Congenital ; Heart Failure ; Heart Ventricles ; Heparin ; Hospitalization ; Humans ; Infant, Newborn* ; Isolated Noncompaction of the Ventricular Myocardium ; Male ; Milrinone ; Mothers ; Muscular Diseases ; Myocardial Infarction* ; Neutropenia ; Siblings ; Thromboembolism

Since the outbreak of the enterovirus 71 (EV71) infection in Malaysia in 1997, large epidemics of EV71 have occurred in the Asia-Pacific region. Many children and infants have died from serious neurological complications during these epidemics, and EV71 infection has become a serious public health problem in these areas. EV71 infection causes hand, foot and mouth disease (HFMD) in children, and usually resolves spontaneously. However, EV71 occasionally involves the central nervous system (CNS), and induces diverse neurological complications such as brainstem encephalitis, aseptic meningitis, and acute flaccid paralysis. Among those complications, brainstem encephalitis is the most critical neurological manifestation because it can cause neurogenic pulmonary hemorrhage/edema leading to death. The characteristic clinical symptoms such as myoclonus and ataxia, cerebrospinal fluid (CSF) pleocytosis, and brainstem lesions on magnetic resonance imaging, in conjunction with the skin rash of HFMD and the isolation of EV71 from a stool, throat-swab, or CSF sample are typical findings indicating CNS involvement of EV71 infection. Treatment with intravenous immunoglobulin and milrinone are recommended in cases with severe neurological complications from EV71 infection, such as brainstem encephalitis. Despite the recent discovery of receptors for EV71 in human cells, such as the scavenger receptor B2 and P-selection glycoprotein ligand 1, it is not known why EV71 infection predominantly involves the brainstem. Recently, 3 companies in China have completed phase III clinical trials of EV71 vaccines. However, the promotion and approval of these vaccines in various countries are problems yet to be resolved.
Ataxia ; Brain Stem ; Central Nervous System ; Cerebrospinal Fluid ; Child ; China ; Encephalitis ; Enterovirus A, Human ; Enterovirus* ; Exanthema ; Glycoproteins ; Hand, Foot and Mouth Disease ; Humans ; Immunoglobulins ; Infant ; Leukocytosis ; Magnetic Resonance Imaging ; Malaysia ; Meningitis, Aseptic ; Milrinone ; Myoclonus ; Neurologic Manifestations ; Paralysis ; Public Health ; Pulmonary Edema ; Receptors, Scavenger ; Vaccines

Severe portopulmonary hypertension (PPHT) is considered a contraindication for liver transplantation (LT) because of the associated high mortality and poor prognosis. We report the case of a 57-year-old cirrhotic woman with severe PPHT (mean pulmonary artery pressure [mPAP] > 65 mmHg), who underwent a successful living donor LT. Intra-operative use of inhaled iloprost, milrinone, dobutamine, and postoperative use of inhaled nitric oxide and oral sildenafil failed to lower the pulmonary artery pressure (PAP). The patient responded only to nitroglycerin and drainage of massive ascites. Meticulous intra-operative volume control, which included minimizing blood loss and subsequent transfusion, was carried out. The use of vasopressors, which may have elevated the PAP, was strictly restricted. Intra-operative PAP did not show an increase, and the hemodynamics was maintained within relatively normal range, compared to the preoperative state. The patient was discharged without any complications or related symptoms.
Ascites ; Dobutamine ; Drainage ; Female ; Hemodynamics ; Humans ; Hypertension* ; Iloprost ; Liver Transplantation* ; Living Donors ; Middle Aged ; Milrinone ; Mortality ; Nitric Oxide ; Nitroglycerin ; Prognosis ; Pulmonary Artery ; Reference Values ; Sildenafil Citrate

BACKGROUND: Milrinone increases intracellular adenosine 3',5'-cyclic monophosphate concentration and enhances vascular relaxation. Nuclear factor-kappa B (NF-kB) plays a key role in inflammatory responses during ischemia-reperfusion (I/R) injury. We aimed to investigate the effect of milrinone on the inflammatory responses and NF-kB activation in renal I/R injury in mice. METHODS: Thirty C57BL/6 mice were allocated into 3 groups. In group S (n = 10), only right nephrectomy was done. In group C (n = 10), the left kidney was subjected to 30 min of ischemia after right nephrectomy. In group M (n = 10), milrinone (5 microg/kg) was administered before ischemia. After 24 hours of reperfusion, the serum creatinine was measured, kidney samples were obtained for histology, and expressions of NF-kB and proinflammatory cytokines were analyzed. RESULTS: In group C, the serum creatinine concentration was markedly elevated, compared with group S. Creatinine concentration in group M was also elevated, but it was significantly lower than that in group C. Histologic evidence of renal damage was severe in group C, but it was improved in group M. In groups C and M, expression of NF-kB, tumor necrosis factor-alpha (TNF-alpha), intercellular adhesion molecule-1 (ICAM-1), monocyte chemoattractant protein-1 (MCP-1) and macrophage inflammatory protein-2 (MIP-2) mRNA increased significantly compared with group S (P < 0.05). But group M showed a lower expression of NF-kB, TNF-alpha, ICAM-1, MCP-1 and MIP-2 mRNA than group C (P < 0.05). CONCLUSIONS: Milrinone treatment attenuates the renal inflammatory response and activation of NF-kB, resulting in improvement of renal function and tissue injury.
Adenosine ; Animals ; Chemokine CCL2 ; Creatinine ; Cytokines ; Intercellular Adhesion Molecule-1 ; Ischemia ; Kidney ; Macrophages ; Mice* ; Milrinone* ; Nephrectomy ; NF-kappa B* ; Relaxation ; Reperfusion ; Reperfusion Injury* ; RNA, Messenger ; Tumor Necrosis Factor-alpha

Pulmonary aspiration of gastric contents is one of the most frightening complications during anesthesia. Although pulmonary aspiration of gastric contents in general surgical patients is not common and resulting long-term morbidity and mortality are rare, severe hypoxemia and other sequelae of pulmonary aspiration continue to be reported. We report a case of massive aspiration of gastric contents during induction of general anesthesia, resulting in cardiac arrest due to severe pulmonary hypertension and myocardial infarction. Sustained cardiac arrest and shock that did not respond the conventional resuscitation was successfully treated using milrinone. The patient was discharged without complications in 20 days.
Anesthesia ; Anesthesia, General ; Anoxia ; Heart Arrest ; Humans ; Hypertension, Pulmonary ; Milrinone* ; Mortality ; Myocardial Infarction ; Respiratory Aspiration of Gastric Contents ; Resuscitation ; Shock ; Shock, Cardiogenic*

BACKGROUND AND OBJECTIVES: Milrinone is often used in children to treat acute heart failure and prevent low cardiac output syndrome after cardiac surgery. Due to the lack of studies on the long-term milrinone use in children, the objective of this study was to assess the safety and efficacy of the current patterns of milrinone use for > or =3 days in infants and children with heart diseases. SUBJECTS AND METHODS: We retrospectively reviewed the medical records of patients aged <13 years who received milrinone for > or =3 days from January 2005 to December 2012. Patients' characteristics including age, sex, height, weight, and body surface area were recorded. The following parameters were analyzed to identify the clinical application of milrinone: initial infusion rate, maintenance continuous infusion rate, total duration of milrinone therapy, and concomitantly infused inotropes. The safety of milrinone was determined based on the occurrence of adverse events such as hypotension, arrhythmia, chest pain, headache, hypokalemia, and thrombocytopenia. RESULTS: We assessed 730 admissions (684 patients) during this period. Ventricular septal defects were the most common diagnosis (42.4%) in these patients. Milrinone was primarily used after cardiac surgery in 715 admissions (97.9%). The duration of milrinone treatment varied from 3 to 64.4 days (> or =7 days in 149 admissions). Ejection fraction and fractional shortening of the left ventricle improved in patients receiving milrinone after cardiac surgery. Dose reduction of milrinone due to hypotension occurred in only 4 admissions (0.5%). Although diverse arrhythmias occurred in 75 admissions (10.3%), modification of milrinone infusion to manage arrhythmia occurred in only 3 admissions (0.4%). Multivariate analysis indicated that the development of arrhythmia was not influenced by the pattern of milrinone use. CONCLUSION: Milrinone was generally administered for > or =3 days in children with heart diseases. The use of milrinone for > or =3 days was effective in preventing low cardiac output after cardiac surgery when combined with other inotropes, suggesting that milrinone could be safely employed in pediatric patients with heart diseases.
Arrhythmias, Cardiac ; Body Surface Area ; Cardiac Output, Low ; Chest Pain ; Child ; Diagnosis ; Headache ; Heart Diseases* ; Heart Failure ; Heart Septal Defects, Ventricular ; Heart Ventricles ; Humans ; Hypokalemia ; Hypotension ; Infant ; Medical Records ; Milrinone* ; Multivariate Analysis ; Off-Label Use* ; Retrospective Studies ; Thoracic Surgery ; Thrombocytopenia

Enterovirus 71 has been recognized as being highly central nervous system (CNS) involved and presents with diverse neurologic manifestations. Brainstem encephalitis is the most common neurologic manifestation of CNS involvement by enterovirus 71, and manifests myoclonus, ataxia, tremor, and autonomic dysfunction such as pulmonary hemorrhage. Here we report a 31-month-old girl with enterovirus 71 brainstem encephalitis presenting with pulmonary hemorrhage and acute heart failure. The patient was admitted to emergency department of our hospital due to high-grade fever, vomiting, myoclonus, and tremor 4 days after hand, foot and mouth disease. Four hours after admission, the patient presented with pulmonary hemorrhage and acute heart failure. CSF analysis demonstrated white blood cell 60/mm3, red blood cell 1/mm3, protein 43.0 mg/dL, and glucose 149 mg/dL. Despite aggressive management including intravenous immunoglobulin, milrinone and empirical antimicrobial therapy, the patient died due to uncontrolled pulmonary hemorrhage and shock in 15 hours after admission at emergency department. In stool specimen obtained from the patient, enterovirus 71 (subgenotype C4a) was detected. This case suggests that an early diagnosis of central nervous system involvement in patient with enterovirus 71 infection is vital because brainstem encephalitis resulting from enterovirus 71 infection can rapidly progress to the critical state of disease.
Ataxia ; Brain Stem* ; Central Nervous System ; Child, Preschool ; Early Diagnosis ; Emergency Service, Hospital ; Encephalitis* ; Enterovirus* ; Erythrocytes ; Female ; Fever ; Glucose ; Hand, Foot and Mouth Disease ; Heart Failure* ; Hemorrhage* ; Humans ; Immunoglobulins ; Leukocytes ; Milrinone ; Myoclonus ; Neurologic Manifestations ; Shock ; Tremor ; Vomiting

OBJECTIVETo investigate the characteristics of circulatory disturbance and treatment of severe hand-foot-and-mouth disease (HFMD).

METHODThe clinical characteristics, laboratory findings, therapy and outcome of 22 severe HFMD patients were retrospectively analyzed.

RESULTAll the 22 severe HFMD patients came from the countryside. All these patients had encephalitis. Fifteen cases had myocardial injury. All had symptoms of sympathetic excitation and 17 cases had hypertension [(128 ± 16)/(81 ± 14) mm Hg (1 mm Hg = 0.133 kPa)]. Fourteen cases had exacerbation with rapid decline of blood pressure [(61 ± 12)/(33 ± 12) mm Hg]. In cardiorespiratory failure stage, 13 patients had neurogenic pulmonary edema accompanied by circulatory failure and 12 cases had a lower glasgow scores (less than 7). Myocardial injury and ECG change were found in some cases. Inotropic and pressor drugs were given in patients with circulatory collapse. Five cases received fluid resuscitation due to refractoriness to inotropic drugs. Nine patients received blood purification. Seventeen survived and 5 cases died due to circulatory failure.

CONCLUSIONCirculation failure of severe HFMD is the main cause of death. Early and appropriate circulation support is very important to reduce mortality.

Child, Preschool ; China ; epidemiology ; Combined Modality Therapy ; Female ; Hand, Foot and Mouth Disease ; complications ; mortality ; therapy ; Humans ; Immunoglobulins, Intravenous ; administration & dosage ; therapeutic use ; Infant ; Intensive Care Units, Pediatric ; Male ; Milrinone ; administration & dosage ; therapeutic use ; Multiple Organ Failure ; etiology ; mortality ; Pulmonary Edema ; etiology ; mortality ; Respiration, Artificial ; Retrospective Studies ; Shock ; etiology ; mortality ; Treatment Outcome

BACKGROUND: Hemodynamic derangement during off-pump coronary artery bypass surgery (OPCAB) is mainly attributed to impaired filling and diastolic dysfunction. An elevated ratio of the mitral velocity to the early-diastolic velocity of the mitral annulus (E/e' > 15) is a relatively new indicator of diastolic function, and this was reported to be associated with impaired hemodynamics during OPCAB. We investigated the efficacy of milrinone on the perioperative hemodynamics and short term outcomes of patients with an E/e' > 15 and who underwent OPCAB. METHODS: The patients were randomly allocated into either group C (control, n = 31) or group M (n = 31) and they were treated with the same amount of either normal saline or milrinone (0.5 microg/kg/min) without bolus loading after completion of internal mammary artery harvest until the end of operation. Hemodynamic measurements were recorded after the induction of anesthesia (T1), 5 min after starting each distal anastomosis of the left anterior descending artery (T2), left circumflex artery (T3) and right coronary artery (T4), and 5 min after sternum closure (T5). RESULTS: The mixed venous oxygen saturation (SvO2) was lower through T2-T4 compared to the baseline value in both groups, while the degree of the decrease was significantly less in group M than that in group C. The other hemodynamic variables, the operative data and the postoperative outcomes were similar between the two groups. CONCLUSIONS: Intraoperative infusion of milrinone did not significantly improve the perioperative hemodynamics and the subsequent short term outcomes for the patients with preexisting diastolic dysfunction as represented by an elevated E/e' value, although it reduced the degree of decrease of the SvO2 during OPCAB.
Anesthesia ; Arteries ; Coronary Artery Bypass, Off-Pump ; Coronary Vessels ; Hemodynamics ; Humans ; Mammary Arteries ; Milrinone ; Oxygen ; Sternum

Tetralogy of Fallot (TOF) assumes its' most severe form when accompanied by pulmonary atresia (PA). Preserving the patent ductus arteriosus to maintain pulmonary blood flow is life-saving for patients with this congenital heart disease. Milrinone, a selective phosphodiesterase III inhibitor, is a potent vasodilator. Here, we report the successful use of milrinone for a newborn infant with TOF and PA for keeping the ductus arteriosus open and thereby maintaining pulmonary circulation. Milrinone is a useful drug because of its inotropic, lusitropic, and pulmonary vasodilating effects, in addition to its ability to keep the ductus arteriosus open and its relatively mild side-effects. Case series and comparative studies will be needed in the future to verify the effectiveness of this drug.
Cyclic Nucleotide Phosphodiesterases, Type 3 ; Ductus Arteriosus ; Ductus Arteriosus, Patent ; Heart Diseases ; Humans ; Infant, Newborn ; Milrinone ; Pulmonary Atresia ; Pulmonary Circulation ; Tetralogy of Fallot