Pediatric Cardiology

The Utility of Echocardiography in Pediatric Patients with Structurally Normal Hearts and Suspected Endocarditis

The Utility of Echocardiography in Pediatric Patients with Structurally Normal Hearts and Suspected Endocarditis. Kelly P, Hua N, Madriago EJ, Holmes KW, Shaughnessy R, Ronai C. Pediatr Cardiol. 2020 Jan;41(1):62-68. doi: 10.1007/s00246-019-02222-z. Epub 2019 Oct 31. PMID: 31673735 Similar articles Select item 31654097   Take Home Points: A single positive blood culture without other major or minor Modified Duke’s Criteria (MDC) and no prior history of congenital heart disease has a positive predictive value of 0 for infectious endocarditis (IE) Two positive cultures without other criteria also has a very low PPV MDC should be used to assess the clinical probability of IE; if low, a TTE is not recommended due to poor diagnostic yield Commentary from Dr. Jared Hershenson (Greater Washington DC), section editor of Pediatric Cardiology Journal Watch:  While sonographic findings are part of the major criteria for MDC, they are usually meant to supplement clinical judgment when there is a higher pretest probability of IE. The sensitivity and specificity in adults is not great for IE, and the ACC/AHA calls TTE an inadequate screening tool. This study was a retrospective chart review to determine the diagnostic yield of TTE in children with suspected IE and structurally normal hearts and no prior history of IE. 300 patients were included. Charts were reviewed to determine whether patients met any MDC prior to the echocardiogram. Clinical IE was determined if patients were treated with 4-6 weeks of IV antibiotics. Positive TTE findings were defined as mass, abscess, thrombus or new significant valvular regurgitation. Over the 10 year study period, 10/300 (3.3%) had positive TTE findings. Of those 10 patients, 8 were treated for IE with 2 false positives that the authors detail. Of the 290 with negative TTE, 3 were diagnosed with IE. These were all teenagers with poorer echo images and clinical features strongly suggestive of IE. See tables 3 and 4.  98 patients (33%) had 2 positive blood cultures; 7 were diagnosed with IE, with one likely having a false positive TTE and the other 6 having some MDC. The PPV of 2 positive cultures and no MDC risk factors was 0.071. 46 patients (15.3%) had only 1 positive blood culture. The PPV of 1 positive culture and no additional MDC risk factors was 0. The PPV of those that met MDC prior to TTE was 0.86. See table 5. There was pretty clear data that those with 1 or 2 positive blood cultures and no other MDC risk factors have a very low likelihood of IE and a positive echo. Misdiagnosis of IE obviously has increased morbidity (need for CVL and antibiotics) and TTE can have false positives. Therefore, use of MDC should be strongly recommended prior to obtaining a TTE. A few caveats/limitations include poor documentation of various vascular and immunologic findings in their chart review. Additionally, while presence of CHD is a risk factor, this study does not address the risk/PPV in this population.  

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New Screening Tool for Aortic Root Dilation in Children with Marfan Syndrome and Marfan-Like Disorders

New Screening Tool for Aortic Root Dilation in Children with Marfan Syndrome and Marfan-Like Disorders. Wozniak-Mielczarek L, Sabiniewicz R, Nowak R, Gilis-Malinowska N, Osowicka M, Mielczarek M. Pediatr Cardiol. 2020 Jan 31. doi: 10.1007/s00246-020-02307-0. [Epub ahead of print] PMID: 32006082 Similar articles Select item 20301322   Take Home Points: Aortic root ratio may be another helpful screening measurement for aortic root dilation in patients with suspected connective tissue disorders This study did not assess normal patients or patients with other etiologies for aortic root dilation (such as BAV) Whether cumulative changes such as rate of change or significant change would improve morbidity/mortality or be useful for surgical intervention remains to be seen Commentary from Dr. Jared Hershenson (Greater Washington DC), section editor of Pediatric Cardiology Journal Watch:  Similar to nearly all echo measurements in children, the aortic diameter is affected by a patient’s age, sex, and BSA. Z-scores are used to allow for the necessary adjustments in order to determine if something is within the normal range. There are currently 3 main z-score nomograms used. Based on a premise that z-score calculations are time consuming and impractical, this study aimed to introduce a simple screening method to identify aortic root dilation. The authors retrospectively analyzed 193 patients with Marfan Syndrome (MFS) or Marfan-like disorders (including Ehlers-Danlos, Loeys-Dietz, ectopia lentis, neonatal Marfan, MASS phenotype, and Marfanoid habitus). Marfanoid habitus was defined as a constellation of symptoms similar to MFS but did not fully meet Ghent criteria. The mean age was 12 years. The aortic root (sinuses of Valsalva only) was measured in the parasternal long axis view using both the leading edge in end-diastole and the inner edge in mid-systole. The aortic root ratio (ARr) was calculated as the aortic root diameter (mm) divided by the height (cm) multiplied by 100. This was compared to the 2 measurement techniques and z-scores using all 3 nomograms.   28-31 % of the patients had aortic root dilation as expressed by z-score (z > 2) depending on which nomogram was used. 11 patients (5.79%) had inconsistent results (dilated vs. non-dilated) when comparing z-score nomograms (Gautier vs. Cantinotti only). The mean value for ARr was 18.1 vs. 17.8 for the leading edge vs. inner edge measurements. The optimal cut-off for ARr was > 18.7 with resulted in a sensitivity of 88-100% and specificity of 94-98% (Table 7). The authors further assessed the subgroup in whom the ARr failed to identify aortic root dilation. They found the results were inconsistent using the 3 different nomograms. Notably, a detailed table shows that all of the z-scores of this group were either just below or just above 2. ROC curves showed an ARr > 18 yielded a sensitivity of 100% for the Petterson and Cantinotti nomograms and > 18.7 for the Gautier nomogram. Tables 10 and 11 show the high values of PPV, NPV and accuracy of ARr using the various cut-offs. The authors make a reasonable argument that extremely thin or obese patients may have inaccurate z-scores, and based on previous data that height is more important than BSA for aortic root diameter, the ARr may be useful. There are however a few major caveats to this study. First, ARr only assess the aortic root at the sinuses. It will therefore not be useful in those with significant annular dilation or ascending aorta dilation, and we know that surgical decision making often entails valve sparing vs. non-sparing aortic root replacement. Additionally, the Marfan-like subgroup was large and may not represent a normal population for which this measurement has not been validated. They also did not include a BAV/aortic root dilation group, or other groups with CHD such as TOF or d-TGA s/p ASO, which may have a different form of aortic root dilation than the genetic connective tissue disorder group. Finally, the claim that nomograms are difficult to use may be a bit spurious, given that many reporting software and measurement packages on the echo equipment may automatically calculate z-scores. It is interesting to note that there may be variation depending on the nomogram used, so echo readers should keep this in mind based on the specific software algorithms used. However, most of these patients would have routine follow up, so a “missed” diagnosis when the z-score is 1.9 is probably not too clinically relevant. Since this was a one-time measurement study, further research on the ARr over time would be necessary, since rate of change is also a key factor in decision making.  

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Imaging of the pulmonary vasculature in congenital heart disease without gadolinium contrast: Intraindividual comparison of a novel Compressed SENSE accelerated 3D modified REACT with 4D contrast-enhanced magnetic resonance angiography

Imaging of the pulmonary vasculature in congenital heart disease without gadolinium contrast: Intraindividual comparison of a novel Compressed SENSE accelerated 3D modified REACT with 4D contrast-enhanced magnetic resonance angiography. Pennig L, Wagner A, Weiss K, Lennartz S, Grunz JP, Maintz D, Laukamp KR, Hickethier T, Naehle CP, Bunck AC, Doerner J. J Cardiovasc Magn Reson. 2020 Jan 23;22(1):8. doi: 10.1186/s12968-019-0591-y. PMID: 31969137 Free PMC Article Similar articles Select item 31974688   Take Home Points: A novel 3D non-contrast-enhanced magnetic resonance angiography (modified REACT-non-CE-MRA) is a good alternative for the visualization of the pulmonary arteries and pulmonary veins in congenital heart disease patients. Compared to 4D contrast-enhanced magnetic resonance angiography, modified REACT-non-CE-MRA offers a better image quality. Comment from Dr. Inga Voges (Kiel, Germany), section editor of Pediatric Cardiology Journal Watch: In this cardiovascular magnetic resonance study the authors applied a new 3D Relaxation-Enhanced Angiography without Contrast and Triggering (modified REACT-non-CE-MRA) to visualize the pulmonary arteries in congenital heart disease (CHD) patients and compared the novel technique with the standard non-ECG-triggered time-resolved 4D contrast-enhanced magnetic resonance angiography (4D CE-MRA). 25 patients with known or suspected CHD were included and images were analysed independently by two radiologists. Measurements were taken at seven distinct measurement points, namely main pulmonary artery, left and right pulmonary artery, right superior and inferior pulmonary vein as well as left superior and inferior pulmonary vein. Furthermore, image quality was assessed using a four-point scale including the parameters sharpness, presence of pulsation artifacts, and anatomic delineation. Out of 25 patients, 23 had a CHD including atrial septal and ventricular septal defect, transposition of the great arteries, tetralogy of Fallot and pulmonary atresia. Regarding the interobserver agreement, the modified REACT-non-CE-MRA had a higher agreement for the pulmonary veins compared to the 4D CE-MRA. For the pulmonary arteries interobserver agreement was comparable between the modified REACT-non-CE-MRA and the 4D CE-MRA. 4D CE-MRA showed larger diameters for all measurement points and this was demonstrated to be significant for the pulmonary arteries but not the pulmonary veins. 4D CE-MRA image quality scores wore lower compared to the scores for the modified REACT-non-CE-MRA (Figures 3 and 5). This study has nicely demonstrated that a novel non-contrast MRA technique, the so-called modified REACT-non-CE-MRA, has the potential to be a good alternative to CE-MRA techniques in CHD patients.

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Fontan-Associated Liver Disease: Spectrum of Disease in Children and Adolescents

Fontan-Associated Liver Disease: Spectrum of Disease in Children and Adolescents. Rathgeber SL, Guttman OR, Lee AF, Voss C, Hemphill NM, Schreiber RA, Harris KC. J Am Heart Assoc. 2020 Jan 7;9(1):e012529. doi: 10.1161/JAHA.119.012529. Epub 2020 Jan 4. PMID: 31902322 Free PMC Article Similar articles Select item 31852418 Select item 31130285   Take Home Points: Fontan-associated liver disease begins in childhood. Liver stiffness measured by transient elastography is associated with splenomegaly and time since Fontan operation. Comment from Dr. Inga Voges (Kiel, Germany), section editor of Pediatric Cardiology Journal Watch: Fontan-associated liver disease is an increasingly important complication and already starts in childhood. In this study the authors evaluated 76 pediatric Fontan patients with a median age of 11.7 years. In all patients an echocardiogram, laboratory studies, transient elastography and an abdominal ultrasound were performed. Furthermore, the aspartate transaminase (AST) to platelet ratio index (APRI) was calculated. 17 patients underwent additional liver biopsy. The authors found a significant correlation between the time since Fontan operation as well as splenomegaly and liver stiffness measured by transient elastography (see figure). In addition, the presence of splenomegaly was found to be associated with low platelets, AST and alkaline phosphatase. Liver biopsy did not demonstrate severe changes in this relatively young cohort, but mild-to-moderate histopathologic liver changes were found in all patients. There was no significant difference regarding liver stiffness between patients with a dominant left and right ventricle. The results are very interesting, but the study also clearly demonstrates that further research in Fontan-associated liver disease is needed.  

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Imaging of the pulmonary vasculature in congenital heart disease without gadolinium contrast: Intraindividual comparison of a novel Compressed SENSE accelerated 3D modified REACT with 4D contrast-enhanced magnetic resonance angiography

Imaging of the pulmonary vasculature in congenital heart disease without gadolinium contrast: Intraindividual comparison of a novel Compressed SENSE accelerated 3D modified REACT with 4D contrast-enhanced magnetic resonance angiography. Pennig L, Wagner A, Weiss K,...

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