Pediatric Cardiology

Risk factors and lifelong impact of community-acquired pneumonia in congenital heart disease. Evers PD, Farkas DK, Khoury M, Olsen M, Madsen NL. Cardiol Young. 2020 Dec 9:1-6. doi: 10.1017/S1047951120004254. PMID: 33292879   Take Home Points: In pneumonia hospitalization. mortality was elevated above the comparison population with a 30-day mortality rate ratio of 1.31 (95% confidence interval: 1.00–1.73). Adults with CHD are at elevated risk of pneumonia hospitalizations and pneumonia-associated mortality. This risk is further elevated in those with severe CHD and extracardiac defects. The cumulative incidence of pneumonia hospitalization was higher for adults with CHD (hazard ratio 1.90; 95% confidence interval: 1.74–2.06) than the comparison cohort. CHD individuals with severe/univentricular subtypes demonstrate a heightened risk compared to the non-CHD cohort (hazard ratio: 2.35; 95% confidence interval: 1.94–2.84), as well as compared to those with mild/moderate CHD (hazard ratio: 1.28; 95% confidence interval: 1.07–1.53).   Commentary from Dr. Manoj Gupta (New York, USA), section editor of Pediatric & Fetal Cardiology Journal Watch: A countrywide cohort study was performed to calculate the relative risk and cumulative incidence of pneumonia hospitalizations and resultant 30-day mortality amongst the adult CHD population, matched 1:10 with non-CHD persons by gender, age, and adjusted for comorbidities. Those with CHD have a twofold   risk of a community-acquired pneumonia-related hospitalizations compared to age- and sex-matched members of the general population. Cumulative incidence of community-acquired pneumonia-related hospitalization in adults with CHD and a general population comparison cohort, with death as competing risk. General population cohort matched on age and gender. CHD = congenital heart disease.   In addition, CHD patients experienced a longer hospitalization period prior to discharge. This highlights the reality that admissions in this population, even when unrelated to their CHD, can lead to greater incremental risk and cost, both direct and indirect, when compared to the general population.   

Lymphatic Disorders and Management in Patients with Congenital Heart Disease. Tomasulo CE, Chen JM, Smith CL, Maeda K, Rome JJ, Dori Y. Ann Thorac Surg. 2020 Dec 26:S0003-4975(20)32169-X. doi: 10.1016/j.athoracsur.2020.10.058. PMID: 33373590   Take Home Points: Congenital heart disease can lead to significant lymphatic complications such as chylothorax, plastic bronchitis, protein losing enteropathy and ascites. Recent improvements in lymphatic imaging and the development of new lymphatic procedures can help alleviate symptoms and improve outcomes. In addition to optimization of the cardiac circulation and medical management, new minimally invasive lymphatic interventional procedures and lymphatic directed surgical procedures are now available and should be utilized to treat patients with these disorder.   Commentary from Dr. Manoj Gupta (New York, USA), section editor of Pediatric & Fetal Cardiology Journal Watch: Magnetic resonance lymphangiography is an important imaging modality for both the peripheral and central lymphatic systems. It can be used as a screening tool for lymphatic abnormalities and has good spatial resolution. T2-weighted MR lymphangiography has demonstrated differences in the lymphatic systems of patients after cavopulmonary anastomoses compared to patients with non-single ventricle CHD. After single ventricle palliation, T2 imaging has shown Thoracic Duct dilation, lymphangiectasia, lymphatic collateralization and tissue edema. After Fontan procedure, patients who developed PLE or plastic bronchitis appear to have statistically significantly larger Thoracic Duct compared to those without such complications.   Lymphatic abnormalities were classified into 4 types (Figure 1).     Figure 1: Classification of T2 Thoracic Lymphatic Abnormalities (A) Type 1: no significant T2 abnormality in mediastinum or neck. (B) Type 2: increased abnormal signal within bilateral supraclavicular region. (C) Type 3: extension into mediastinum. (D) Type 4: further extension into interstitium of lungs. Copyright ©2019, the Radiological Society of North America.   The higher-grade types had significantly longer postoperative hospital stays and duration of effusions, mortality, orthotopic heart transplant (OHT), ECMO, plastic bronchitis and acute Fontan takedown only occurred in patients with type 4, indicating a poor prognosis for patients in this group. This imaging should be used as a screening tool for thoracic lymphatic abnormalities in all single ventricle patients prior to the Fontan operation.   Noncontrast magnetic resonance lymphangiography   Intrahepatic Dynamic contrast magnetic resonance lymphangiography is a new imaging modality designed to assess liver lymphatic anatomy and flow and is the modality of choice for patients with PLE and ascites, Intranodal Dynamic contrast magnetic resonance lymphangiography is one of the more recent imaging techniques and is the modality of choice for central lymphatic flow disorders and Intramesenteric Dynamic contrast magnetic resonance lymphangiography is another new imaging modality that is now available for certain forms of PLE.   In all patients with suspected lymphatic abnormalities, cardiac catheterization should be performed to assess hemodynamics and determine reversible causes of lymphatic failure, such as SVC or branch pulmonary artery stenosis. Single ventricle patients after the Fontan procedure with no obstruction through the Fontan pathway can undergo creation or recreation of a Fontan fenestration to help reduce pressures. Lymphatic interventions can be separated into those that serve to decompress the lymphatic system, such as lymphovenous anastomosis (LVA), surgical or percutaneous thoracic duct decompression, and those that target exclusion of the abnormal lymphatic channels, including selective lymphatic duct embolization, placement of covered stents in the thoracic duct, ethiodized oil lymphatic embolization, liver lymphatic embolization, and thoracic duct embolization or ligation.   

Method comparison of HPLC-ninhydrin-photometry and UHPLC-PITC-tandem mass spectrometry for serum amino acid analyses in patients with complex congenital heart disease and controls Michel M, Salvador C, Wiedemair V, Adam MG, Laser KT, Dubowy KO, Entenmann A, Karall D, Geiger R, Zlamy M, Scholl-Bürgi S. Metabolomics. 2020 Dec 15;16(12):128. doi: 10.1007/s11306-020-01741-8. PMID: 33319318 Free PMC article.   Take Home Points: Precision and accuracy were acceptable for photometry and UHPLC (ultra-high-performance liquid)-tandem mass spectrometry in the analysis of serum amino acid concentrations. Although amino acid concentrations differed significantly between photometry and tandem mass spectrometry both methods showed similar patterns of serum amino acid concentrations in patients and controls.   Commentary from Dr. Inga Voges (Kiel, Germany), chief section editor of Pediatric & Fetal Cardiology Journal Watch: Serum amino acids are associated with oxidative stress and inflammation in Fontan patients. In this method comparison study, the authors compared photometry with UHPLC (ultra-high-performance liquid)-tandem mass spectrometry to analyze serum amino acids in Fontan patients and healthy controls.   20 Fontan patients with a double inlet left ventricle and their matched controls were included. Overall, 24 amino acids and their serum concentrations were compared. Precision and accuracy were acceptable for both methods. Concentrations for many amino acids differed significantly between the two methods in both Fontan patients and controls (Figure 1). However, the amino acid concentration pattern was similar between the two methods (Figure 1).   Bland-Altman plots are shown (Figure 2 and 3) and demonstrated a negative trend for the differences that is proportional to the magnitude of the measurement. As explained by the authors, this negative trend indicates that tandem mass spectrometry tends to yield lower concentration than photometry if analyte concentrations are low. If concentrations are high, tandem mass spectrometry tends to yield higher amino acid concentrations than photometry.   The authors conclude that both methods are suitable for pattern recognition of serum amino acid concentrations. Figure 1   Figures 2 and 3    

[et_pb_section fb_built="1" admin_label="section" _builder_version="3.22" custom_padding="||188px|||"][et_pb_row admin_label="row" _builder_version="3.25" background_size="initial" background_position="top_left" background_repeat="repeat" custom_margin="-101px|auto|-208px|auto||"][et_pb_column type="4_4" _builder_version="3.25" custom_padding="|||" custom_padding__hover="|||"][et_pb_text admin_label="Text" _builder_version="3.27.4" background_size="initial" background_position="top_left" background_repeat="repeat" min_height="1797px"] Early school-age cognitive performance post-pediatric heart transplantation Gold A, Bondi BC, Ashkanase J, Dipchand AI.Pediatr Transplant. 2020 Dec;24(8):e13832. doi: 10.1111/petr.13832. Epub 2020 Oct 26.PMID: 33105067   Take Home Points In this small (n= 25), but well controlled study of post-transplant pediatric patients, intellectual, academic, and perceptual-motor domain scores were low average while memory abilities were average. Clinical DSM-5 psychological diagnosis was present in >50% of the patients studied. Presence of a congenital heart disease and/or psychological diagnosis predicted worse neuropsychological domain scores. Close neuropsychological monitoring of these patients are needed to maximize long-term outcomes. Commentary from Dr. Clifford Cua (Columbus Ohio USA), section editor of Pediatric/Fetal Cardiology Journal Watch: Previous literature, using heterogeneous methodologies, has documented increased developmental delay, neuropsychological deficits in cognition, and decreased academic achievement in pediatric heart transplant patients with an incidence ranging from 10 – 50%. The goal of this study was to evaluate a non-biased patient sample under uniform conditions and time intervals to better understand the neuropsychological outcomes in these patients. Patients who underwent cardiac transplantation < 2 years of age were recruited. Multiple neuropsychological assessment tools (Table 1) were performed till entry into grade school. Scores were designated average (90 - 109), low average (80 – 89), well below average (70 – 79), and significantly poorer than age expectations (< 70). The study was performed from January 2014 to October 2018. Patients were excluded if they had been assessed before or could not follow up for testing. Baseline demographics as well as clinical data were recorded. Thirty-one patients underwent transplantation during the study period and 25 qualified for the study (18 females, 7 males). Median age at transplantation was 0.71 years and age at testing was 6.7 years. Fourteen patients had CHD and 11 had cardiomyopathy. 72% had neurological issues and 68% had sensory issues pre- or post-transplant. General intellect, academic, and perceptual motor indices were in the low normal range, whereas memory indices were in the normal range (Figure 1 – 3). Fourteen (56%) patients met DSM-5 criteria for a clinical diagnosis (intellectual disability mild 20%, learning disability 20%, language disorder 8%, and ADHD 12%). Another 8 (32%) patients were deemed at risk for developing issues. There was a negative correlation between prior neurological issues (stroke, seizure, microcephaly, or other) and full-scale intelligent quotient, verbal comprehension, working memory, visual learning, verbal story learning, and short delay verbal story memory. There was a negative correlation with CHD and full-scale intelligent quotient, working memory, and fluid reasoning. This study reinforces the need to closely follow up these patients from a neuropsychological standpoint so that early intervention can be performed to maximize long-term outcomes. Though not surprising, it also reinforces that patients with a congenital heart disease diagnosis or prior neurological issues are at increased risk for poorer neuropsychological outcomes. Though the study was small, from a single institution, and had a limited follow up time period, it has the benefits of being complete in its assessment of the patient population.           [/et_pb_text][/et_pb_column][/et_pb_row][et_pb_row _builder_version="4.7.4" _module_preset="default" custom_margin="83px|auto||auto||"][et_pb_column type="4_4" _builder_version="4.7.4" _module_preset="default"][et_pb_image src="https://uv0pl4on9b-flywheel.netdna-ssl.com/wp-content/uploads/2021/01/Picture1.png" title_text="Picture1" _builder_version="4.7.4" _module_preset="default" min_height="729px"][/et_pb_image][/et_pb_column][/et_pb_row][et_pb_row _builder_version="4.7.4" _module_preset="default"][et_pb_column type="4_4" _builder_version="4.7.4" _module_preset="default"][et_pb_image src="https://uv0pl4on9b-flywheel.netdna-ssl.com/wp-content/uploads/2021/01/Picture2.png" title_text="Picture2" _builder_version="4.7.4" _module_preset="default"][/et_pb_image][/et_pb_column][/et_pb_row][et_pb_row _builder_version="4.7.4" _module_preset="default"][et_pb_column type="4_4" _builder_version="4.7.4" _module_preset="default"][et_pb_image src="https://uv0pl4on9b-flywheel.netdna-ssl.com/wp-content/uploads/2021/01/Picture3.png" title_text="Picture3" _builder_version="4.7.4" _module_preset="default"][/et_pb_image][/et_pb_column][/et_pb_row][et_pb_row _builder_version="4.7.4" _module_preset="default"][et_pb_column type="4_4" _builder_version="4.7.4" _module_preset="default"][et_pb_image src="https://uv0pl4on9b-flywheel.netdna-ssl.com/wp-content/uploads/2021/01/Picture4.png" title_text="Picture4" _builder_version="4.7.4" _module_preset="default"][/et_pb_image][/et_pb_column][/et_pb_row][et_pb_row _builder_version="4.7.4" _module_preset="default"][et_pb_column type="4_4" _builder_version="4.7.4" _module_preset="default"][et_pb_image src="https://uv0pl4on9b-flywheel.netdna-ssl.com/wp-content/uploads/2021/01/Picture5.png" title_text="Picture5" _builder_version="4.7.4" _module_preset="default"][/et_pb_image][/et_pb_column][/et_pb_row][/et_pb_section]