Clinical Utility of Echocardiography for the Diagnosis and Prognosis in Children with Bronchopulmonary Dsyplasia

BACKGROUND: Bronchopulmonary dysplasia (BPD) may result in chronic pulmonary artery hypertension and right ventricular (RV) dysfunction. Various echocardiographic assessments of RV dysfunction have been used to determine whether echocardiographic measurements of premature infants with BPD could provide sensitive measures of RV function that correlates with BPD severity. METHODS: Twenty-eight control subjects without BPD (non BPD group), 28 patients with mild BPD, 11 patients with moderate BPD, and six patients with severe BPD underwent echocardiograms with standard measurement such as ejection fraction by M-mode, tricuspid regurgitation pressure gradient, myocardial performance index (MPI) derived from pulse Doppler, and tissue Doppler imaging (TDI) measurements. BPD severity was classified by the NICHD/NHLBI/ORD workshop rating scale. Twenty-eight control subjects without BPD (non BPD group), 28 patients with mild BPD, 11 patients with moderate BPD, and six patients with severe BPD underwent echocardiograms with standard measurement such as ejection fraction by M-mode, tricuspid regurgitation pressure gradient, myocardial performance index (MPI) derived from pulse Doppler, and TDI measurements. BPD severity was classified by the NICHD/NHLBI/ORD workshop rating scale. RESULTS: None of the standard echocardiographic findings was significantly different between the control group and BPD groups. However, mean septal TDI-MPI of the severe BPD group (0.68 ± 0.06) was significantly (p < 0.01) higher than that of the non-BPD (0.58 ± 0.10) or the mild BPD group (0.59 ± 0.12). In addition, mean RV TDI-MPI of the severe BPD group (0.71 ± 0.13) was significantly (p < 0.05) higher than that of the non-BPD group (0.56 ± 0.08) or the mild BPD group (0.60 ± 0.125). Linear regression showed a good correlation between the severity of BPD and RV TDI-MPI (p = 0.01, R = 0.30) or septal TDI-MPI (p = 0.04, R = 0.24). CONCLUSION: Echocardiographic evaluation of RV function based on an assessment of RV TDI-MPI can provide RV dysfunction parameter in premature infants with BPD.

Hashimoto thyroiditis with an unusual presentation of cardiac tamponade in Noonan syndrome / 소아과

Noonan syndrome is an autosomal dominant, multisystem disorder. Autoimmune thyroiditis with hypothyroidism is an infrequent feature in patients with Noonan syndrome. A 16-year-old boy was admitted because of chest discomfort and dyspnea; an echocardiogram revealed pericardial effusion. Additional investigations led to a diagnosis of severe hypothyroidism due to Hashimoto thyroiditis. The patient was treated with L-thyroxine at 0.15 mg daily. However, during admission, he developed symptoms of cardiac tamponade. Closed pericardiostomy was performed, after which the patient's chest discomfort improved, and his vital signs stabilized. Herein, we report a case of an adolescent with Noonan syndrome, who was diagnosed with Hashimoto thyroiditis with an unusual presentation of cardiac tamponade.

Total Anomalous Pulmonary Venous Return in Siblings

Total anomalous pulmonary venous return (TAPVR) is a rare and critical congenital vascular anomaly that requires an early operation. However, initial symptoms of TAPVR may be non-specific, and cardiovascular findings may be minimal. The heart may not be enlarged and there is often no cardiac murmur. Without cardiac murmur, these symptoms are similar to those of respiratory distress syndrome in newborns. Therefore, a high degree of suspicion and an early diagnosis of TAPVR are important. This condition generally occurs without a family history and has a low recurrence rate, but several familial cases, including siblings, have been reported worldwide. Additionally, several chromosomal or gene abnormalities associated with TAPVR have been reported. In the case presented here, two brothers with a 6-year age gap were diagnosed with TAPVR. Surgery was performed without cardiac or neurological complications. This is the first report on TAPVR in siblings in Korea.

Efficacy of Electroencephalographic Monitoring for the Evaluation of Intracranial Injury during Extracorporeal Membrane Oxygenation Support in Neonates and Infants / 대한구급학회지

BACKGROUND: Neurological complications are a serious concern during extracorporeal membrane oxygenation (ECMO) support in neonates and infants. However, evaluating brain injury during ECMO has limitations. Herein, we report our experience with bedside electroencephalographic monitoring during ECMO support and compared this to post-ECMO brain imaging studies and immediate neurologic outcomes. METHODS: We retrospectively reviewed the data for 18 children who underwent ECMO. From these subjects, we reviewed the medical records of 10 subjects who underwent bedside EEG monitoring during ECMO support. We collected data on patient demographics, clinical details of the ECMO course, electroencephalographic monitoring, brain imaging results, and neurologic outcomes. RESULTS: The median age was 4 months (range: 7 days-22 months), the median weight was 5 (3.6-12) kg, and the median length of ECMO therapy was 86 (27-206) hours. Eight patients (80%) were weaned successfully, and seven (70%) survived to discharge. Those with normal to mildly abnormal electroencephalographic findings had non-specific to mildly abnormal brain computed tomography findings and no neurologic impairment. Those patients with a moderately to severely abnormal electroencephalograph had markedly abnormal brain computed tomography findings and remained neurologically impaired. CONCLUSIONS: Normal electroencephalographic findings are closely related to normal or mild neurologic impairment. Our results indicate that electroencephalographic monitoring during ECMO support can be a feasible tool for evaluating brain injury although further prospective studies are needed.

Effect of Indomethacin Treatment in Full-term Infants with Symptomatic Patent Ductus Arteriosus / 대한주산의학회잡지

PURPOSE: Indomethacin has been reported as the prophylaxis and initial treatment of preterm infants with patent ductus arteriosus (PDA). However, there was controversy over indomethacin treatment in full-term infants with symptomatic PDA. Therefore, we evaluate the effect of indomethacin as a treatment of full-term infants with symptomatic PDA. METHODS: A retrospective study was performed to evaluate the effectiveness of indomethacin in full-term infants who had birth weight > or =2,500 g and a gestational age > or =37 weeks with symptomatic PDA at Chonnam National University Hospital between January 2007 and December 2009. According to responsiveness of indomethacin, we classified them into three groups: 1) complete responder which were completely closed after indomethacin treatment, 2) partial responder which were incompletely closed but symptoms were improved, 3) non responder which were conducted surgical ligation because did not respond. RESULTS: Among the total 29 full-term infants treated with indomethacin, 13 (44.8%) were complete responder, 8 (27.6%) were partial responder, and 8 (27.6%) were non responder. There were no significant differences in birth weight, narrow diameter of PDA, and dose of indomethacin between three groups. However, the age at initiation of treatment using indomethacin of complete (4.8+/-4.5 days, P=0.03) and partial responder (6.3+/-2.0 days, P=0.04) were earlier than those of non responder (13.8+/-8.1 days). CONCLUSION: Indomethacin can expect an effective treatment of PDA in full-term infants prior to surgical ligation.

Transvenous proximal closure of large congenital coronary arteriovenous fistula using the single Amplatzer vascular plug in a 3-year-old girl / 소아과

Congenital coronary arteriovenous fistulas (CCAFs) are rare coronary artery abnormalities in which blood is shunted into a cardiac chamber or great vessel. If the fistula itself is large and tortuous, it is generally recommended to occlude the fistula to prevent several complications. In approaches of transcatheter occlusion, the transvenous approach is preferred over the transarterial approach. The transvenous approach would enable the cannulation of a relatively larger catheter or sheath without potential damage to the femoral vessels or normal coronary arteries, which can occur in the transarterial approach. The transvenous approach may also minimize the blind pouch after releasing the devices. Herein, we report the success of transvenous proximal closure of a CCAF using an Amplatzer vascular plug (AVP) in a 3-year-old patient with cardiomegaly. Complete occlusion was achieved by a single AVP and thrombus formation of the distal aneurysmal portion of the fistula. We suggest that this strategy of closing the proximal end with a dilated fistula using a single AVP by the transvenous approach may be a good option in treating CCAFs in a young child.

Right ventricular failure in congenital heart disease / 소아과

Despite developments in surgical techniques and other interventions, right ventricular (RV) failure remains an important clinical problem in several congenital heart diseases (CHD). RV function is one of the most important predictors of mortality and morbidity in patients with CHD. RV failure is a progressive disorder that begins with myocardial injury or stress, neurohormonal activation, cytokine activation, altered gene expression, and ventricular remodeling. Pressure-overload RV failure caused by RV outflow tract obstruction after total correction of tetralogy of Fallot, pulmonary stenosis, atrial switch operation for transposition of the great arteries, congenitally corrected transposition of the great arteries, and systemic RV failure after the Fontan operation. Volume-overload RV failure may be caused by atrial septal defect, pulmonary regurgitation, or tricuspid regurgitation. Although the measurement of RV function is difficult because of many reasons, the right ventricle can be evaluated using both imaging and functional modalities. In clinical practice, echocardiography is the primary mode for the evaluation of RV structure and function. Cardiac magnetic resonance imaging is increasingly used for evaluating RV structure and function. A comprehensive evaluation of RV function may lead to early and optimal management of RV failure in patients with CHD.

Partial anomalous pulmonary venous connection with intact atrial septum in a child with ventricular septal defect: a case report / 소아과

Partial anomalous pulmonary vein connection (PAPVC) is a rare congenital abnormal cardiac defect involving the pulmonary veins draining into the right atrium (RA) directly or indirectly by venous connection. Ninety percent of PAPVCs are accompanied by atrial septal defect (ASD). To our knowledge, there is no previous report of PAPVC with ventricular septal defect (VSD) without ASD in Korea, and in this paper, we report the first such case. A 2-day-old girl was admitted into the Chonnam National University Hospital for evaluation of a cardiac murmur. An echocardiogram revealed perimembranous VSD without ASD. She underwent patch closure of the VSD at 5 months of age. Although the VSD was completely closed, she had persistent cardiomegaly with right ventricular volume overload, as revealed by echocardiography. Three years later, cardiac catheterization and chest computed tomography revealed a PAPVC, with the right upper pulmonary vein draining into the right SVC. Therefore, correction of the PAPVC was surgically performed at 3 years of age. We conclude that it is important to suspect PAPVC in patients with right ventricular volume overload, but without ASD.

Mosaic Ring Chromosome 6 in an Infant with Significant Patent Ductus Arteriosus and Multiple Congenital Anomalies

The clinical features of ring chromosome 6 include central nervous system anomalies, growth retardation, facial dysmorphism and other congenital anomalies. Ring chromosome 6 occurs rarely and manifests as various phenotypes. We report the case of mosaic ring chromosome 6 by conventional karyotyping in a 7-day-old male infant diagnosed with a large patent ductus arteriosus (PDA) with hypoplasia of aortic valve and aortic arch. These have not been previously reported with ring chromosome 6. He recovered from heart failure symptoms after ligation of the PDA. He showed infantile failure to thrive and delayed milestone in a follow-up evaluation. To the best of our knowledge, this is the first report of a Korean individual with ring chromosome 6 and hemodynamically significant PDA.

Recurrent Plastic Bronchitis in a Child with 2009 Influenza A (H1N1) and Influenza B Virus Infection

Plastic bronchitis is an uncommon disorder characterized by the formation of bronchial casts. It is associated with congenital heart disease or pulmonary disease. In children with underlying conditions such as allergy or asthma, influenza can cause severe plastic bronchitis resulting in respiratory failure. A review of the literature showed nine cases of plastic bronchitis with H1N1 including this case. We report a case of a child with recurrent plastic bronchitis with eosinophilic cast associated with influenza B infection, who had recovered from plastic bronchitis associated with an influenza A (H1N1) virus infection 5 months previously. To the best of our knowledge, this is the first case of recurrent plastic bronchitis related to influenza viral infection. If patients with influenza virus infection manifest acute respiratory distress with total lung atelectasis, clinicians should consider plastic bronchitis and early bronchoscopy should be intervened. In addition, management for underlying disease may prevent from recurrence of plastic bronchitis.

Change in Blood Pressure and Pulse Pressure in Preterm Infants After Treatment of Patent Ductus Arteriosus With Indomethacin

BACKGROUND AND OBJECTIVES: Patent ductus arteriosus (PDA) is associated with increased morbidity and mortality in premature infants. Therefore, an early diagnosis and treatment of a hemodynamically significant PDA are very important. A widened pulse pressure is considered to be a well known clinical sign of a PDA in older infants and children; however, whether this is also applicable in the case of preterm infants remains to be confirmed. The aims of this study were to investigate the change in blood pressure (BP) before and after medical treatment of a PDA with indomethacin and to evaluate if the change in the pulse pressure in preterm infants with a medically treated PDA could be used as a reliable clinical predictor of a hemodynamically significant PDA. SUBJECTS AND METHODS: Between January 2005 and June 2009, a retrospective analysis was performed in preterm infants with a hemodynamically significant PDA (PDA group, n=72) and preterm infants without a PDA (control group, n=72) at the Chonnam National University Hospital Neonatal Intensive Care Unit. The PDA was closed by treatment with indomethacin. The BP was compared between the two groups over the seven days after the first dose of indomethacin. RESULTS: In preterm infants with a hemodynamically significant PDA, the mean systolic (55.1+/-6.0 mmHg) and diastolic BPs (31.4+/-6.2 mmHg) were lower than those in the controls (mean systolic BP 58.0+/-6.4 mmHg, mean diastolic BP 34.7+/-6.0 mmHg) before indomethacin treatment. When the ductus arteriosus was successfully closed by indomethacin treatment, there was a gradual increase in both the systolic and diastolic BPs without any change in the pulse pressure. CONCLUSION: The results of this study show that a widened pulse pressure is not a useful clinical sign of a hemodynamically significant PDA in preterm infants. However, low systolic and diastolic BPs may be useful clinical signs of a hemodynamically significant PDA in preterm infants. If the systolic and diastolic BP is low, a PDA should be considered and echocardiography should be performed for early diagnosis and treatment.

Final height of patients with congenital heart disease / 소아과

PURPOSE: Growth impairment is usually observed in children with congenital heart disease (CHD). Studies on the final height and weight of this population are insufficient. Our aim was to evaluate the final height of children with CHD and to identify the relationship between CHD and growth. METHODS: We recorded the values of final height and weights of 105 CHD patients (age, <19 years) who visited Chonnam National University Hospital between November 2000 and March 2009, and we reviewed the medical records. RESULTS: The mean values of weight and height of male CHD patients were significantly lower than those of the normal subjects. Patients with severe growth impairment (below the third percentile of normal) included 5 males (8.6%) and 4 females (8.5%) with height less than normal and 9 males (15.5%) and 8 females (17.0%) with weight less than normal. The mean growth of the patients in the cyanotic heart disease group was lower than that of normal subjects, but a statistically significant difference was noted in the weights of males. In a comparative study based on the type of CHD, a significant difference was noted in weights of males. Patients with patent ductus arteriosus and those with tetralogy of Fallot had the highest and lowest mean values of weight, respectively. CONCLUSION: Growth impairment was more evident in children with CHD than normal children. Patients should be treated during an optimal time frame. Thus, CHD patients should be treated if follow-up studies indicate growth impairment.

The characteristic laboratory findings of non-responsiveness to intravenous immunoglobulin in children with Kawasaki disease / 소아과

PURPOSE: Although intravenous immunoglobulin (IVIG) treatment is an effective first-line treatment for Kawasaki disease, 10-20% of the patients develop persistent fever or coronary artery complications. Medical records of Kawasaki disease patients were reviewed to assess the characteristic laboratory findings of IVIG nonresponsiveness. METHODS: We reviewed the clinical records of 118 children with Kawasaki disease who were treated at the Chonnam National University Hospital from March 2003 to February 2008. The laboratory findings of the IVIG-responder group (n=110) and the IVIG-nonresponder group (n=8) were compared at admission day and at 48 hours and 14 days after IVIG administration. RESULTS: At admission, the level of creatine kinase (CK) was lower (P = 0.03) and that of total protein was higher (P < 0.01) in the nonresponders than in the responders. At 48 hours after IVIG administration, the white blood cell (WBC) count (P = 0.04) and neutrophil% (P < 0.01) was higher in the nonresponders than in the responders. The neutrophil% (P < 0.01) and CK (P = 0.01) level at admission was lower than that at 48 hours after IVIG administration in the responders; this decrease was not as apparent in the nonresponders. CONCLUSION: IVIG nonresponders have lower CK and higher total protein levels at admission and higher WBC count and neutrophil% at 48 hours after IVIG administration. The decrease in the neutrophil% and CK level between at admission and at 48 hours after IVIG administration is remarkably higher in responders than in nonresponders.

Secondary Subaortic Stenosis after Patch Closure of Subarterial Ventricular Septal Defect

Subaortic stenosis usually occurs without a previous heart operation, however, it can occur after heart surgery as well, with a condition known as a secondary subaortic stenosis (SSS). SSS has been reported after surgical repair of several congenital heart defects. There are only a few recorded cases of SSS after repair of ventricular septal defect (VSD). Here we report a rare case of SSS that occurred 3 years after surgical repair of subarterial VSD. A follow-up echocardiogram is essential for detecting SSS caused by the newly developed subaortic membrane in patients who had cardiac surgery.

Late adverse reactions to iopromide (Ultravist(R)) diagnosed by the patch test: a case report / 소아과

Iodinated contrast media (CM) can cause immediate and late reactions. We treated a patient with a recurrent generalized maculopapular rash and a fever that occurred within two days of exposure to iodinated CM, iopromide (Ultravist(R)), for chest computed tomography. We performed skin testing including prick tests, intradermal tests, and patch tests. Our findings indicated a late skin reaction to Ultravist(R) in addition to cross-reactions to other iodinated CM such as ioversol (Optiray(R)), iohexol (Iobrix(R)), and iobitridol (Xenetix(R)). In this study, we report the case of a patient diagnosed with a late adverse reaction to Ultravist(R) in addition to cross-reactions to other iodinated CM.

Herpes zoster complicated by deep vein thrombosis : a case report / 소아과

Varicella zoster virus (VZV) causes two diseases: Varicella, a generalized, primary infection, and herpes zoster (zoster), a secondary infection caused by latent VZV reactivation. Zoster can also be caused by latent VZV reactivation after a varicella vaccination. The complications associated with varicella include cutaneous infections, which are the most common, as well as pulmonary and neurological involvement. However, a deep venous thrombosis (DVT) has been rarely described as a varicella-associated complication. Here, we describe the case of a child with varicella zoster who developed a DVT that completely resolved after intravenous acyclovir and subcutaneous low-molecular-weight heparin treatment.

Herpes zoster complicated by deep vein thrombosis : a case report / 소아과

Varicella zoster virus (VZV) causes two diseases: Varicella, a generalized, primary infection, and herpes zoster (zoster), a secondary infection caused by latent VZV reactivation. Zoster can also be caused by latent VZV reactivation after a varicella vaccination. The complications associated with varicella include cutaneous infections, which are the most common, as well as pulmonary and neurological involvement. However, a deep venous thrombosis (DVT) has been rarely described as a varicella-associated complication. Here, we describe the case of a child with varicella zoster who developed a DVT that completely resolved after intravenous acyclovir and subcutaneous low-molecular-weight heparin treatment.

Fatal plastic bronchitis with eosinophilic casts in a previously healthy child / 소아과

Plastic bronchitis is a rare disease characterized by the recurrent formation of branching mucoid bronchial casts that are large and more cohesive than those that occur in ordinary mucus plugging. Casts may vary in size and can be spontaneously expectorated, but some require bronchoscopy for removal. Plastic bronchitis can therefore present as an acute life-threatening emergency if obstruction of the major airways occurs. Three of 22 reported patients with eosinophilic casts were fatal, with death due to central airway obstruction. Here, we report a child with no history of atopy, allergy, or congenital heart disease who was diagnosed with plastic bronchitis with eosinophilic casts. Although he was administered intravenous (iv) antibiotics; iv corticosteroids; and a vigorous pulmonary toilet regimen, including chest physiotherapy and routine bronchoscopic removal of casts, he had brain death secondary to hypoxic brain damage. Plastic bronchitis can be fatal when casts obstruct the major airways, as in the present case. Clinicians should intervene early if a patient exhibits signs and symptoms consistent with plastic bronchitis.

Fatal plastic bronchitis with eosinophilic casts in a previously healthy child / 소아과

Plastic bronchitis is a rare disease characterized by the recurrent formation of branching mucoid bronchial casts that are large and more cohesive than those that occur in ordinary mucus plugging. Casts may vary in size and can be spontaneously expectorated, but some require bronchoscopy for removal. Plastic bronchitis can therefore present as an acute life-threatening emergency if obstruction of the major airways occurs. Three of 22 reported patients with eosinophilic casts were fatal, with death due to central airway obstruction. Here, we report a child with no history of atopy, allergy, or congenital heart disease who was diagnosed with plastic bronchitis with eosinophilic casts. Although he was administered intravenous (iv) antibiotics; iv corticosteroids; and a vigorous pulmonary toilet regimen, including chest physiotherapy and routine bronchoscopic removal of casts, he had brain death secondary to hypoxic brain damage. Plastic bronchitis can be fatal when casts obstruct the major airways, as in the present case. Clinicians should intervene early if a patient exhibits signs and symptoms consistent with plastic bronchitis.

A case of subcutaneous paragonimiasis presented with pleural effusion / 소아과

Paragonimiasis is a parasitic infection that occurs following the ingestion of infectious Paragonimus metacercariae from crabs or crayfish. Pulmonary paragonimiasis is the most common clinical manifestation of this infection, but several ectopic paragonimiasis cases have also been reported. Among them, cases of subcutaneous paragonimiasis are rare, especially in children. We report a case of subcutaneous paragonimiasis of the right abdominal wall with pleural effusion with hepatic involvement and without abnormal pulmonary infiltration in a boy aged 2 years and 5 months. He had eaten soybean sauce-soaked freshwater crabs (kejang) 6 months prior to complaining of right abdominal wall distension. On evaluation, right pleural effusion without abnormal pulmonary infiltration was detected, as well as blood eosinophilia, an elevated serum IgE level, pleural fluid eosinophilia and a positive enzyme-linked immunosorbent assay that detected P. westermani antibody in the serum. Thoracentesis, praziquantel administration, and excision of subcutaneous lesions were performed. After treatment, the eosinophil count and serum IgE level were decreased, and the subcutaneous lesions did not recur. The frequency of paragonimiasis has decreased recently, but it is still prevalent in Korea. Paragonimiasis should be suspected if pleural fluid eosinophilia is associated with blood hypereosinophilia and a high level of serum IgE; however clinicians should obtain a thorough history of travel and food habits.