December

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    

Long-Term Fate of the Truncal Valve.  Gellis L, Binney G, Alshawabkeh L, Lu M, Landzberg MJ, Mayer JE, Mullen MP, Valente AM, Sleeper LA, Brown DW.J Am Heart Assoc. 2020 Nov 17;9(22):e019104. doi: 10.1161/JAHA.120.019104. Epub 2020 Nov 9.PMID: 33161813   Take Home Points: Long-term rates of truncal valve intervention are significant - by 20 years post-operative period, a quarter of patients with truncus arteriosus underwent truncal valve intervention (repair or replacement). Moderate or greater initial truncal valve dysfunction and single coronary ostium were associated with subsequent need for truncal valve intervention. Larger truncal valve root z-score is associated with significant truncal valve regurgitation and may identify a subset of patients at risk for truncal valve dysfunction over time.     Commentary from Dr. Manoj Gupta (New York, USA), section editor of Pediatric & Fetal Cardiology Journal Watch: This is a retrospective chart review study from 1985 to 2016 at Boston Children’s Hospital, and a total of 244 patients underwent initial TA repair at BCH.     Of these patients, 170 met the criteria for analysis. Overall, 9% (n=15/170) of patients underwent concomitant truncal valve surgery at time of initial repair. Two thirds (n=10/15) of these patients had a quadricuspid valve.  Eleven patients had greater than mild preoperative truncal valve regurgitation, and the remaining 4 patients had moderate or severe truncal valve stenosis. Three patients also had concomitant left coronary artery intervention: unroofing, translocation, and removal of fibrous tissue at the ostium. Of the patients with moderate or greater initial regurgitation, 15 (79%) had a quadricuspid valve.   Overall, 123 patients (83%) underwent at least one surgical or catheter-based intervention during follow-up. Freedom from any surgical reintervention at 1, 5, 10, and 20 years was 90.0%, 50.0%, 21.0%, and 6.0%.   Quadricuspid truncal valve (n=45/140) and concomitant truncal valve surgery at initial repair (n=11/148) were univariate risk factors for truncal valve intervention, a truncal root z-score of ≥5 had a significantly higher odds of developing moderate or greater truncal valve regurgitation     During follow-up, 30 patients (20%) had at least one surgical intervention on the truncal valve, 24 of whom were from the group without concomitant truncal valve surgery (first intervention on the truncal valve occurred subsequently during follow-up) (Figure 1). Among those with only subsequent truncal intervention, 16 underwent repair first and 8 underwent replacement without prior repair. Of note, those who underwent replacement were older (median age, 18.3 [range, 1.1–23.0] years versus 8.3 [range, 2.3–16.8] years; P=0.04) and with larger aortic root size at time of replacement.   Four patients had >1 valve repair, and 3 patients went on to have a second valve replacement during follow-up. Of the 11 long-term survivors with concomitant truncal valve surgery at initial repair, 6 went on to have truncal valve reintervention during follow-up.   During follow-up, 10 patients (7%) underwent truncal root reduction (2 at time of surgery for conduit exchange without truncal valve intervention, 4 at time of truncal valve replacement, and 4 at time of truncal valve re- pair). No patients experienced aortic dissection.   

Low-dose prostaglandin E1 is safe and effective for critical congenital heart disease: is it time to revisit the dosing guidelines? Daniel Vari 1, Wendi Xiao 2, Shashank Behere 3, Ellen Spurrier 3, Takeshi Tsuda 3, Jeanne M Baffa 3 Cardiol Young. 2021 Jan;31(1):63-70. doi: 10.1017/S1047951120003297. Epub 2020 Nov 3. PMID: 33140712; DOI: 10.1017/S1047951120003297   Take Home Points: Prostaglandin E1 at an initial and maintenance dose of 0.01 μg/kg/minute was sufficient to maintain ductal patency in 83% in this study, instead of the standard starting dose of prostaglandin E1 is 0.05 μg/kg/minute. Starting low-dose prostaglandin E1 at 0.01 μg/kg/minute is a safe and effective therapy for critical CHD. Patients with pulmonary obstruction were more likely to require higher doses than patients with systemic obstruction. Postnatally diagnosed patients with systemic obstruction are also at a higher risk of dose escalation than prenatally diagnosed infants. The incidence of respiratory depression requiring mechanical ventilation was low and was mostly seen in premature infants.      Commentary from Dr. Manoj Gupta (New York, USA), section editor of Pediatric & Fetal Cardiology Journal Watch: Of the 153 eligible patients, 127 (83%) were started and maintained on a prostaglandin E1 dose of 0.01 μg/kg/minute until the end- point. Of the 26 patients who had their doses increased, the final dose was less than 0.05 μg/kg/minute in 15, 0.05 μg/kg/minute in five, and greater than 0.05 μg/kg/minute in two patients. 15 patients had their dose increased due to both echocardiographic findings and clinical factors suggesting ductal constriction. In systemic obstruction patients, these factors included blood pressure gradients, pulse abnormalities between upper and lower extremities, and elevated serum lactate levels. In pulmonary obstruction and inadequate mixing patients, the primary clinical factor driving dose increase was hypoxemia. In six patients, there was an echocardiographic finding of ductal constriction without corresponding clinical signs.   Of the 137 patients analyzed for respiratory depression, 38 (28%) had documented respiratory depression at a dose of 0.01μg/kg/ minute. In 10 of these patients, the respiratory depression was transient and did not merit initiation of respiratory support although four were started on caffeine. Fourteen patients (10%) were started on nasal cannula or high-flow nasal cannula, three patients (2%) were placed on continuous positive airway pressure, and 11 patients (8%) were mechanically ventilated via endotracheal intubation as a result of respiratory depression. Premature infants were more likely to experience respiratory depression (12/18, 67%) than term infants (26/117, 22%, p < 0.001). Mechanical ventilation was also more frequent in premature infants (6/18, 33%) than in term infants (5/117, 4%, p = 0.001).