A Heart Elsewhere: The Unusual Diagnosis of a Congenital Ventricular Diverticulum in the Abdomen.

Congenital left ventricular (LV) diverticulum is a rare condition characterized by the presence of a contractile appendix originating usually from the cardiac apex, but with high variability in location, dimension, and clinical presentation. We describe the diagnostic process and clinical management of an isolated apical diverticulum discovered during fetal life. (Level of Difficulty: Advanced.).

Prenatal Identification of Restrictive and Non-restrictive Ventricular Septal Defects Based on End-Systolic Flow Patterns in the Fetal Aortic Isthmus.

Fetuses with large ventricular septal defects (VSDs) must be closely monitored during the perinatal period. Intrauterine assessment of size of septal defects with bidimensional echocardiography are unreliable. The objective of the present study is to document the contribution of flow pattern analysis in the fetal aortic isthmus for prenatal identification of large non-restrictive VSDs requiring immediate postnatal attention. We conducted a cross-sectional retrospective study of Doppler recordings from patients referred to our Fetal Cardiology Unit and diagnosed with one or multiple VSDs from January 2006 to November 2015. Three groups were defined: (1) large non-restrictive VSDs (n = 11) with postnatal cardiac failure (2) small restrictive VSDs (n = 28) asymptomatic, and (3) absence of cardiac abnormality (n = 66). The Isthmic end-Systolic Index (ISI) was computed to quantify aortic isthmus flow and compared between the three groups. Fetuses with restrictive VSD and control group showed similar ISI: stable at 0.20 ± 0.01 up to 27 weeks of gestation and dropping later due to the fall of end-systolic velocities in the aortic isthmus. By 35 weeks, a brief end-systolic retrograde flow was present, associated with a further fall of ISI down to -0.26 ± 0.05. In contrast, ISI of fetuses with large non-restrictive VSDs were unchanged throughout gestation (0.18 ± 0.06), becoming significantly higher during the last weeks of gestation compared to the two other groups (p < 0.001). In fetuses with VSDs, the ISI measurement from the aortic isthmus Doppler flow during the second half of gestation is a reliable predictor of a large non-restrictive defect with risk of major postnatal clinical impact.

Toward the integration of global longitudinal strain analysis in the assessment of neonatal aortic coarctation? A preliminary study.

BACKGROUND: Coarctation of the aorta (CoA) is still challenging to diagnose in neonates with patent ductus arteriosus (PDA). Speckle tracking echocardiography allows reliable analysis of myocardial deformation in newborns and seems to provide important insides into regional changes in patients with left ventricular (LV) outflow tract obstruction. AIMS: To assess the interest of LV global longitudinal strain (GLS) measurement for predicting CoA in neonates with PDA and prenatal suspicion. METHODS: Prospective single-center study. Twenty-two newborns with prenatal suspicion of CoA were included. All newborns were evaluated in the first 12 hours of life. To assess the feasibility and the reproducibility of GLS, 14 healthy full-term newborns with PDA (group 3) were screened. CoA was diagnosed when DA closed, according to usual echocardiographic criteria. RESULTS: Six neonates developed CoA after DA closure (group 1) whereas 16 did not (group 2). Mean gestational age and birth weight were not different between the groups. GLS measurements were possible in 100%. Intra- and inter-observer variability of strain measurements was acceptable. GLS values were significantly lower in neonates who developed CoA (P=0.015). To predict CoA, cut-off value of -17.42% gave the best compromise for sensitivity (83%) and specificity (72%). Aortic arch dimensions were modestly correlated with strain values. The presence of a bicuspid aortic valve was not associated with significant lower GLS values. CONCLUSION: LV GLS analysis is a feasible and reproducible echocardiographic technique in newborns with PDA. Newborns who will develop CoA seem to have lower values of GLS than healthy neonates. Further studies are needed to confirm these preliminary results.

First Case of Q Fever Endocarditis Involving the Melody® Transcatheter Pulmonary Valve in an Afebrile Child.

In this article we report the first case of Q fever endocarditis in a 13 years old child with a percutaneous pulmonary Melody® valve. The patient had a new onset of Melody valve dysfunction associated with the combination of hepatosplenomegaly and pancytopenia but was afebrile. Although blood cultures were negative, we have further investigated in the direction of infective endocarditis by performing PCR detection and the serology of C. burnetii which were positive. A combination antibiotic therapy with doxycycline and hydroxychloroquine was started with good clinical evolution. Our case emphasizes the fact that any Melody valvular dysfuntion should be considered as a potential infective endocarditis despite the absence of typical bacterial features.

Marfan Sartan: a randomized, double-blind, placebo-controlled trial.

AIMS: To evaluate the benefit of adding Losartan to baseline therapy in patients with Marfan syndrome (MFS). METHODS AND RESULTS: A double-blind, randomized, multi-centre, placebo-controlled, add on trial comparing Losartan (50 mg when <50 kg, 100 mg otherwise) vs. placebo in patients with MFS according to Ghent criteria, age >10 years old, and receiving standard therapy. 303 patients, mean age 29.9 years old, were randomized. The two groups were similar at baseline, 86% receiving ß-blocker therapy. The median follow-up was 3.5 years. The evolution of aortic diameter at the level of the sinuses of Valsalva was not modified by the adjunction of Losartan, with a mean increase in aortic diameter at the level of the sinuses of Valsalva of 0.44 mm/year (s.e. = 0.07) (-0.043 z/year, s.e. = 0.04) in patients receiving Losartan and 0.51 mm/year (s.e. = 0.06) (-0.01 z/year, s.e. = 0.03) in those receiving placebo (P = 0.36 for the comparison on slopes in millimeter per year and P = 0.69 for the comparison on slopes on z-scores). Patients receiving Losartan had a slight but significant decrease in systolic and diastolic blood pressure throughout the study (5 mmHg). During the study period, aortic surgery was performed in 28 patients (15 Losartan, 13 placebo), death occurred in 3 patients [0 Losartan, 3 placebo, sudden death (1) suicide (1) oesophagus cancer (1)]. CONCLUSION: Losartan was able to decrease blood pressure in patients with MFS but not to limit aortic dilatation during a 3-year period in patients >10 years old. ß-Blocker therapy alone should therefore remain the standard first line therapy in these patients.

Prenatal diagnosis of long QT syndrome with the superior vena cava-aorta Doppler approach.

We describe a fetus at 36 weeks with long QT syndrome presenting with variable types of atrioventricular blocks, ventricular premature beats, and torsades de pointes. All these diagnoses were made with the superior vena cava-aorta Doppler approach and confirmed with postnatal electrocardiography.

Right ventricle three-dimensional echography in corrected tetralogy of fallot: accuracy and variability.

AIMS: To evaluate right ventricular (RV) volume and ejection fraction (EF) in adult normal subjects and repaired tetralogy of Fallot (ToF) with 3D trans-thoracic echocardiography (3DE) and a semi-automatic border detection algorithm. METHODS AND RESULTS: Fourteen healthy volunteers and 20 patients with repaired ToF (mean age 31 +/- 14) underwent 3DE and MRI within the same day. Right ventricular end-systolic volume (ESV) and end-diastolic volume (EDV) and EF were measured by two observers using 3DE and compared with MRI measurements. Intra- and interobserver variability of 3DE and agreement between both methods were evaluated using Bland-Altman analysis. Over or underestimation of 3DE in comparison to MRI was assessed using paired t-test. Intra- and interobserver variability of 3DE was excellent with intraclass coefficient of correlation (ICC) ranging from 0.85 to 0.99 and from 0.85 to 0.98, respectively. Three-dimensional echocardiography underestimated ESV and EDV (P < 0.001) but agreement between 3DE and MRI was excellent (ICC = 0.88 and 0.87, respectively). Ejection fraction was 47.7 +/- 7.8 with 3DE and 47.9 +/- 6.7 with MRI, agreement between both methods was good (ICC = 0.72). CONCLUSION: Three-dimensional echocardiography combined to semi-automated quantification software shows fair agreement with MRI for RV volumes and EF measurement in patients with repaired ToF and adequate intra- and interobserver variability. These results suggest applicability for serial follow-up of patients with right heart congenital disease. However, the accuracy of 3DE echo diminishes with larger RV volumes, in part due to current difficulty to include the entire RV in the imaged sector. Technical progress in transducers beam geometry is likely to address this issue.

Validation of an echocardiographic multiparametric strategy to increase responders patients after cardiac resynchronization: a multicentre study.

AIMS: We sought to develop and validate a multiparametric algorithm by applying previously validated criteria to predict cardiac resynchronization therapy (CRT) response in a multicentre study. Thirty per cent of patients treated by CRT fail to respond to the treatment. Although dyssynchrony by echocardiography has been used to improve the selection of patients, the complexity of myocardial contraction has generated a moderate improvement using any of several individual parameters. METHODS AND RESULTS: Two hundred end-stage heart failure patients [NYHA 3-4 and left ventricular ejection fraction (LVEF)<35%] with QRS>120 ms were included. Echocardiography analysis focused on the following parameters: atrioventricular dyssynchrony, interventricular dyssynchrony, and intraventricular dyssynchrony that integrated radial (PSAX M-mode) and longitudinal [tissue Doppler imaging (TDI)] evaluations for spatial (wall to wall) and temporal (wall end-systole to mitral valve opening) dyssynchrony diagnosis. Following CRT implantation, patients were monitored for 6 months with functional and echo evaluations defining responders by a 15% reduction in end-systolic volume. Mean QRS duration and LVEF were 152 +/- 17 ms and 25 +/- 8%. There was a CRT response in 57% of patients, independent of QRS width. Mean prevalence of positive criteria was 34 +/- 8%. Feasibility and variability averages were 81 +/- 20% and 9 +/- 4%. In a single parametric approach, ranges of sensitivities and specificities were 18-65% and 45-84% with a mean of 41% and 66%. A multiparametric approach by focusing on criteria combination decreased the mean rate of false-positive results to 14 +/- 12%, 5 +/- 4%, 2 +/- 2%, and 1 +/- 2% from one to four parameters, respectively. More than three parameters were associated with a specificity above 90% and a positive predictive value above 65%. Reproducibility of this global strategy was 91%. CONCLUSION: A multiparametric echocardiographic strategy based on the association of conventional criteria is a better indicator of CRT response than the existing single parametric approaches.

Presence of ventricular dyssynchrony and haemodynamic impact of right ventricular pacing in adults with repaired Tetralogy of Fallot and right bundle branch block.

AIMS: Late after surgical repair, adults with Tetralogy of Fallot (TOF) commonly present with right ventricular (RV) dysfunction and right bundle branch block (RBBB). We aimed at (i) investigating whether this prolonged RV conduction induced detrimental electromechanical dyssynchrony in both RV and left ventricle (LV) and (ii) determining the acute haemodynamic effects of pacing at different RV sites. METHODS AND RESULTS: A total of 42 adults with surgically repaired TOF and RBBB were investigated by echocardiography. Intra-RV dyssynchrony (IRVD) and intra-left ventricular dyssynchrony (ILVD) were compared with measurements performed in 30 healthy matched control subjects. An acute haemodynamic study was subsequently performed in a subgroup of 10 patients with New York Heart Association functional class II or class III and echocardiographic signs of RV dysfunction. Cardiac index was measured by a thermodilution technique during spontaneous rhythm (SR) and during atrio-synchronized RV pacing at four different sites (infundibulum, apex, septal, and lateral walls). Fifty-five per cent of the patients with repaired TOF demonstrated abnormal RV and/or LV dyssynchrony. We observed an increased IRVD (37 +/- 12 vs. 18 +/- 8 ms; P= 0.02) and ILVD (34 +/- 12 vs. 20 +/- 10 ms; P= 0.04) in TOF patients when compared with control subjects. We did not observe any significant acute improvement in the cardiac output during atrio-synchronized ventricular pacing vs. SR. Similarly, RV pacing did not induce any significant reduction in the QRS duration. CONCLUSION: Some TOF adults with RBBB exhibit biventricular electromechanical dyssynchrony. However, in symptomatic patients with RV dysfunction, atrio-synchronized RV pacing does not induce significant acute haemodynamic improvement.