RAPIDLY DEVELOPING CONSTRICTIVE PERICARDITIS IN AN INFANT SIMULATING MITRAL VALVE OBSTRUCTION

A case is reported of rapidly developing constrictive pericarditis in a 9-month-old infant, apparently the youngest yet described. Unusual features of this case were the failure of the heart size to decrease as expected, and electrocardiographic, roentgenographic, angiocardiographic and hemodynamic data, which indicated “mitral valve obstruction”. These unusual features resulted from the peculiar location of the pericardial constriction over the left ventricle, which took the form of a pericardial cyst hampering left atrial emptying.     

Pericardial involvement at diagnosis in pediatric Hodgkin lymphoma patients

BACKGROUND: Because most cases are clinically silent, the incidence, clinical course, and outcome of pericardial involvement in Hodgkin lymphoma are unknown. METHODS: Records of all patients with newly diagnosed Hodgkin lymphoma treated at our institution between 1991 and 2004 were reviewed. Pericardial involvement was identified by computerized tomography (CT) as focal thickening or nodularity present at the time of diagnosis, and by echocardiography as pericardial effusion. Outcomes measured were incidence of pericardial involvement, relapse-free survival, and overall survival. RESULTS: Thirteen of 273 patients (5%) had pericardial involvement. All patients with pericardial involvement had nodular sclerosing tumors versus 183 of 260 patients without pericardial involvement (P = 0.02); 9 (67%) had a bulky mediastinal mass versus 27% (P = 0.002). Two patients required pericardial drainage to drain very large effusions (n = 2). Both patients were symptomatic with either shortness of breath or superior vena cava syndrome. In the 11 cases that did not undergo surgical drainage, the effusion resolved within days after starting chemotherapy. Two patients experienced distant relapse but underwent successful salvage therapy. All 13 patients remain alive and free of disease at a median follow-up of 9.7 years (range, 1.7-12.9 years) with normal cardiac function. CONCLUSIONS: Pericardial involvement by lymphoma is usually asymptomatic unless accompanied by substantial pericardial effusion. In most cases, pericardial involvement resolves with treatment of the underlying malignancy, but close observation for hemodynamic complications is required. A symptomatic effusion, once treated, does not affect survival.     

Perforation complications of percutaneous central venous catheters in very low birthweight infants

OBJECTIVE: To prospectively survey perforation complications of consecutively inserted percutaneous central venous catheters (PCVC) in very low birthweight (VLBW) infants over a 2 year period. METHODOLOGY AND RESULTS: Three serious perforation complications were encountered in a series of 100 consecutive PCVC. One infant (birthweight 685 g) developed pericardial effusion and fatal cardiac tamponade during the use of a polyurethane PCVC. At autopsy, the pericardial sac contained 8 mL fluid with a glucose concentration of 109 mmol/L and the catheter tip was embedded in the right ventricular wall. The second infant (birthweight 1380 g) showed pleural effusion and transient immobility of the right diaphragmatic leaf after perforation of a similar PCVC into the right pleural cavity. The third perforation, causing subcutaneous oedema, occurred in a 655 g infant who had a silastic PCVC. CONCLUSIONS: The data suggest a 3% incidence for PCVC-associated symptomatic perforation complications and a 1% incidence for fatal perforations, despite a policy of careful placement. The data also indicate that perforation complications occur regardless of the size or material of the PCVC. Proper visualization of the PCVC and vigilant attention to its location is required to prevent these rare but potentially fatal complications.     

ULTRASOUNDCARDIOGRAPHY IN INFANTS AND CHILDREN

Ultrasoundcardiography (UCG) studies have been performed in about three hundred children. The age of the children varied between 1 day and 16 years. Most of the studies have been performed without premedication and all without discomfort. The UCG-technique has been found valuable for demonstration of dilatation of the right ventricle both in cyanotic and acyanotic congenital heart disease. In studies of lesion affecting the tricuspid valve pathological UCG-findings have been obtained in patients with tricuspid atresia and Ebsteins anomaly. The method has also been found useful in evaluation of the state of the mitral valve. Pathological findings have been obtained in congenital mitral stenosis, mitral atresia and other forms of hypoplastic left heart syndrome, in endocardial cushion defects and in mitral regurgitation. The UCG-technique also provides information about the outflow tract of the left ventricle. Pathological findings have been obtained in cases with membraneous subvalvular aortic stenosis and hypertrophic obstructive cardiomyopathy. Pericardial effusion and thrombosis in the right atrium may be demonstrated by this technique. No complications have been observed.     

以反复心包积液为表现的婴儿心脏K-M综合征1例报告

目的探讨心脏K-M综合征的临床特点、诊断及治疗。方法回顾分析1例心脏K-M综合征患儿的临床资料,并复习相关文献。结果患儿,男,出生33天。表现为呼吸急促、颈静脉充盈、心音低顿等。彩色多普勒超声心动图及胸部CT提示大量心包积液。血常规显示血小板计数下降。急诊行心包部分切除引流术,升主动脉根部、上腔静脉、右心耳上缘范围可见6 cm×5 cm硬质包块,固定。右室流出道及肺动脉处见长条形凸起包块,质硬,固定。右心室表面部分瘤体及左侧壁层心包病理显示为Kaposi血管内皮瘤。确诊为心脏K-M综合征。给予类固醇激素治疗。结论心脏K-M综合征是一种罕见疾病,主要根据临床表现、影像学及病理学检查诊断,可给予口服类固醇激素等治疗,必要时手术治疗。     

Massive pericardial effusion due to intrapericardial mixed germ cell tumor in a premature baby

Primary cardiac tumor is uncommon in childhood, with an incidence of 0.06–0.32%, and intrapericardial teratoma represents an exceptional rarity among these entities. Germ cell tumors (GCT) are rare, representing only 1–3% of childhood tumors. Twenty per cent of GCT are malignant and are associated with age and location. Extragonadal involvement accounts for nearly half of the cases. Anterior mediastinum is a common location of malignant germ cell tumors, yet pericardial and aortic adventitia involvement have been rarely reported. Here we report the case of a preterm twin baby boy with intrapericardial mixed germ cell tumor who presented with hydrops fetalis and pericardial effusion.     

Yellow nail syndrome in infancy

Abstract: This report describes an infant with clinical features consistent with the yellow nail syndrome (YNS), a rare autosomal dominant disorder. He presented at birth with congenital lymphoedema and was referred at 6 months of age for investigation of recurrent cough and wheeze. He had clinical and radiological evidence of bilateral pleural effusions and a pericardial effusion. Following a lung biopsy and pericardial window these were shown to be manifestations of his lymphatic abnormality. He also had persisting middle ear effusions causing conductive deafness requiring hearing aids and secondary immunodeficiency requiring regular immunoglobulin infusions.     

Pericardial effusion complicating a percutaneous central venous line in a neonate

A premature infant developed pericardial effusion four days after the insertion of a 25-gauge silastic percutaneous central venous catheter. The effusion contained parenteral nutrition fluid and resolved rapidly after withdrawal of the catheter. Pericardial effusion is a potential complication of percutaneous, as well as surgically placed, central venous catheters.     

Pericardial effusion following hematopoietic stem cell transplantation in children: Incidence,risk factors,and outcomes

PCE is a complication of HSCT that has previously been described in small single‐center studies. This study aimed to assess the frequency of, risk factors for, and outcomes of children with a PCE following HSCT across a large multi‐center cohort. All patients ≤21 years undergoing first HSCT (1/2005‐9/2015) were identified from the Pediatric Health Information System. ICD‐9 codes were used to identify patients with a PCE during or following the transplant encounter. Multivariable modeling assessed risk factors for developing a PCE and assessed the impact of PCE on patient outcome. Of 10 455 included patients, 739 (7.1%) developed a PCE (median 69 days post‐HSCT, interquartile range 33‐165 days). PCE developed more commonly in allogeneic vs autologous HSCT recipients (9.1% vs 2.9%, P < .001). Among allogeneic HSCT recipients, independent risk factors for PCE included thrombotic microangiopathy (AHR 2.94, 95% CI 2.16‐4.00), heart failure (AHR 2.07, 95% CI 1.61‐2.66), PCE pre‐HSCT (AHR 1.92, 95% CI 1.19‐3.09), arrhythmia (AHR 1.76, 95% CI 1.44‐2.16), graft‐versus‐host disease (AHR 1.31, 95% CI 1.05‐1.62), female sex (AHR 1.28, 95% CI 1.07‐1.52), and malignancy (AHR 1.28, 95% CI 1.02‐1.60). Allogeneic HSCT patients with PCE demonstrated worse survival than those without PCE (5‐year survival 50.8% vs 76.9%, P < .001). PCE was independently associated with mortality (AHR 1.96, 95% CI 1.62‐2.37) following allogeneic HSCT and was not impacted by pericardial intervention. PCE occurs more commonly in patients following allogeneic (vs autologous) HSCT and is associated with inferior outcomes.     

Coexistence of Ductal Constriction and Closure of the Foramen Ovale <Emphasis Type="Italic">in Utero</Emphasis>

We report a fetus with an unusual combination of a narrow ductus arteriosus (DA) and foramen ovale. A pregnant mother was referred at 26 weeks of gestation for fetal pericardial effusion. Fetal echocardiography showed pericardial effusion, right atrial enlargement, right ventricular hypertrophy, and tricuspid regurgitation. The DA looked tortuous with S-shaped kinking. The atrial septum primum bulged into the left atrium. Color Doppler did not show any flow across the atrial septum. Cesarean section was performed at 31 weeks of gestation. Admission to intensive care was required after delivery, but the infant gradually improved and was discharged home without any sequela.     

Cardiac tamponade and pericardial effusion due to venous umbilical catheterization

AIM: We present three cases of neonatal cardiac tamponade due to umbilical venous catheterization, a rare, but potentially fatal complication. METHODS: Timely diagnosis was made by echocardiography, and an urgent pericardiocentesis revealed TPN fluid. Perforation of the cardial wall was proven by contrast X-ray showing contrast diffusing into the pericardial space. DISCUSSION: Most frequently, perforation has a delayed course and results from endothelial injury, caused by the hyperosmolar fluids, leading to transmural necrosis and thrombosis. Subsequently, the fluid diffuses transmurally across the myocardium into the pericardium. As migration of the catheter tip can occur, we suggest that its position should be checked immediately after insertion and twice a week thereafter. CONCLUSION: Pericardial effusion and cardiac tamponade should be considered in any infant with a central venous line who develops a rapid, unexplained clinical deterioration. Timely diagnosis and drainage has been proven to be life-saving.     

Neurological features in Gaucher's disease during enzyme replacement therapy

This report describes two patients with Gaucher's disease who had unusual clinical symptoms during enzyme replacement therapy. One patient was a female with type 3 Gaucher's disease. She developed a pericardial effusion at 7 y of age, which contained many Gaucher cells despite enzyme replacement therapy. She died from neurological deterioration during enzyme replacement therapy, despite an improvement in her visceral manifestations. The other patient is a male with type 2 Gaucher's disease, who has achieved long-term survival after being supported by mechanical ventilation and enzyme replacement therapy. While on enzyme replacement therapy at the age of 4 y, he suffered a generalized cutaneous disease which was clinically diagnosed as ichthyosis. Conclusion: These cases suggest that ordinary enzyme replacement therapy is insufficient for some of the non-neurological manifestations of severe types of Gaucher's disease.     

Cardiac tamponade in a pediatric renal transplant recipient on sirolimus therapy

Because of its lack of nephrotoxicity, the use of sirolimus, as an immunosuppressive agent, has increased considerably in solid-organ transplant (Tx) recipients. With its increased use, Tx professionals are encountering a variety of previously unreported side-effects such as angioedema and interstitial pneumonitis. We describe here the case of a pediatric renal Tx recipient who, while receiving sirolimus, developed a large pericardial effusion requiring pericardiocentesis. An extensive workup for an infectious etiology was performed; the only positive result was isolation of adenovirus type 2 from the patient's stool specimen. Following sirolimus dose reduction this child's effusion stabilized and has not recurred. The purpose of this report is to advise health-care professionals caring for Tx recipients about this potentially life-threatening complication associated with sirolimus. The role of adenovirus, if any, in contributing to the development of our patient's pericardial effusion is discussed herein.     

Transient myeloproliferative disorder and eosinophilic pericardial effusion in a down syndrome neonate

Transient myeloproliferative disorder seen in neonates with Down syndrome is often thought to have a benign course. The authors describe the clinical and laboratory profile of a neonate with Down phenotype and transient myeloproliferative disorder with pericardial effusion as co-morbidity. Pericardial fluid analysis showed eosinophils. Pericardial effusion resolved with prednisolone therapy. Regression in hepatosplenomegaly with clearance of blasts was seen by third week of illness. The clinical course suggested a benign infiltration of the pericardium. Presence of eosinophils supports the differentiating capability of the blast cells in transient myeloproliferative disorders.     

Acute Pericarditis in Childhood: A 10-Year Experience

Twenty children, aged 6 months to 13 years, with acute pericarditis admitted between 1987 and 1997 to a university hospital were analyzed retrospectively for their etiology, presentation, management, and prognosis. The most common types of pericarditis were purulent (40%), collagen vascular disease (30%), viral (20%), and neoplastic disease (10%). Most children presented with chest pain, fever, and tachypnea, but cardiac tamponade was not seen in any children. Staphylococcus aureus was the most frequent causative organism of purulent pericarditis and septic arthritis was the most common concurrent infection in the patients. Surgical drainage was performed for 11 cases, 9 underwent subxiphoid pericardial window, and 2 underwent thoracotomy. There was no constrictive pericarditis or reaccumulation of fluid after surgery. Two children died, one of staphylococcal septicemia and the other had a malignant mediastinal tumor. The remaining 18 made a complete recovery. We conclude that subxiphoid pericardial drainage is a simple, safe, and quick procedure and can be done easily in general hospitals by pediatric surgeons. The expensive facilities of cardiac surgeries are not needed.