Dual-energy computed tomography to detect early pulmonary vascular changes in children with sickle cell disease: a pilot study.

Introduction: Pulmonary hypertension (PH) is a rare but fatal complication of sickle cell disease (SCD) that is possibly reversible if treated early. Dual-energy computed tomography (DECT) is a valuable tool for diagnosing PH. We attempted to determine if DECT can detect early signs of PH in children with SCD. Methods: This prospective observational pilot study was conducted at the Geneva University Hospitals and was approved by the local human ethics committee (CCER 2019-01975). A written informed consent was obtained from the patients and/or their legal guardian. Eight children (consisting of five girls and three boys) with homozygous SCD were included in the study. They underwent full cardiological workup using transthoracic echocardiography (TTE) and cardiopulmonary exercise test (CPET), as well as DECT. Results: The median age of the children was 11 years old (range 8-12). All patients exhibited a normal biventricular systo-diastolic function using the TTE. The median tricuspid regurgitant jet velocity value was 2.24 m/s (range 1.96-2.98). Four children were found to have signs of vasculopathy detected on DECT. Of them, two had abnormal screening test results. They both had an increased VE/VCO2 slope during CPET and an increased TVR of >2.5 m/s on TTE. Conclusion: DECT is capable of identifying early signs of pulmonary vascular disease in children with SCD. Further studies are needed to understand the correlation between DECT abnormalities and hemodynamic pulmonary circulation better.

Correlation between Pulmonary Artery Pressure and Vortex Duration Determined by 4D Flow MRI in Main Pulmonary Artery in Patients with Suspicion of Chronic Thromboembolic Pulmonary Hypertension (CTEPH).

Chronic thromboembolic pulmonary hypertension (CTEPH) is one of the causes of pulmonary hypertension (PH) and requires invasive measurement of the mean pulmonary artery pressure (mPAP) during right heart catheterisation (RHC) for the diagnosis. 4D flow MRI could provide non-invasive parameters to estimate the mPAP. Twenty-five patients with suspected CTEPH underwent cardiac MRI. Mean vortex duration (%), pulmonary distensibility, right ventricular volumes and function were measured using 4D flow MRI and cine sequences, and compared with the mPAP measured by RHC. The mPAP measured during RHC was 33 ± 16 mmHg (10−66 mmHg). PH (defined as mPAP > 20 mmHg) was present in 19 of 25 patients (76%). A vortical flow was observed in all but two patients (92%) on 4D flow images, and vortex duration showed good correlation with the mPAP (r = 0.805; p < 0.0001). Youden index analysis showed that a vortex duration of 8.6% of the cardiac cycle provided a 95% sensitivity and an 83% specificity to detect PH. Reliability for the measurement of vortex duration was excellent for both intra-observer ICC = 0.823 and inter-observer ICC = 0.788. Vortex duration could be a useful parameter to non-invasively estimate mPAP in patients with suspected CTEPH.

International registry of congenital porto-systemic shunts: a multi-centre, retrospective and prospective registry of neonates, children and adults with congenital porto-systemic shunts.

BACKGROUND: Congenital portosystemic shunts (CPSS) are rare vascular malformations associated with the risk of life-threatening systemic conditions, which remain underdiagnosed and often are identified after considerable diagnostic delay. CPSS are characterized by multiple signs and symptoms, often masquerading as other conditions, progressing over time if the shunt remains patent. Which patients will benefit from shunt closure remains to be clarified, as does the timing and method of closure. In addition, the etiology and pathophysiology of CPSS are both unknowns. This rare disorder needs the strength of numbers to answer these questions, which is the purpose of the international registry of CPSS (IRCPSS). METHOD: A retrospective and prospective registry was designed using secuTrial® by the ISO certified Clinical Research Unit. Given that a significant number of cases entered in the registry are retrospective, participants have the opportunity to use a semi-structured minimal or complete data set to facilitate data entry. In addition, the design allows subjects to be entered into the IRCPSS according to clinically relevant events. Emphasis is on longitudinal follow-up of signs and symptoms, which is paramount to garner clinically relevant information to eventually orient patient management. The IRCPSS includes also three specific forms to capture essential radiological, surgical, and cardiopulmonary data as many times as relevant, which are completed by the specialists themselves. Finally, connecting the clinical data registry with a safe image repository, using state-of-the-art pseudonymization software, was another major focus of development. Data quality and stewardship is ensured by a steering committee. All centers participating in the IRCPSS have signed a memorandum of understanding and obtained their own ethical approval. CONCLUSION: Through state-of-the-art management of data and imaging, we have developed a practical, user-friendly, international registry to study CPSS in neonates, children, and adults. Via this multicenter and international effort, we will be ready to answer meaningful and urgent questions regarding the management of patients with CPSS, a condition often ridden with significant diagnostic delay contributing to a severe clinical course.

Non-Invasive Cardiac Output Determination Using Magnetic Resonance Imaging and Thermodilution in Pulmonary Hypertension.

Magnetic resonance imaging (MRI) can be used to measure cardiac output (CO) non-invasively, which is a paramount parameter in pulmonary hypertension (PH) patients. We retrospectively compared stroke volume (SV) obtained with MRI (SVMRI) in six localisations against SV measured with thermodilution (TD) (SVTD) and against each other in 24 patients evaluated in our PH centre using Bland and Altman (BA) agreement analyses, linear correlation, and intraclass correlation (ICC). None of the six tested localisations for SVMRI reached the predetermined criteria for interchangeability with SVTD, with two standard deviations (2SD) of bias between 24.1 mL/beat and 31.1 mL/beat. The SVMRI methods yielded better agreement when compared against each other than the comparison between SVMRI and SVTD, with the best 2SD of bias being 13.8 mL/beat. The inter-observer and intra-observer ICCs for COMRI were excellent (inter-observer ICC between 0.889 and 0.983 and intra-observer ICC between 0.991 and 0.999). We could not confirm the interchangeability of SVMRI with SVTD based on the predetermined interchangeability criteria. The lack of agreement between MRI and TD might be explained because TD is less precise than previously thought. We evaluated a new method to estimate CO through the pulmonary circulation (COp) in PH patients that may be more precise than the previously tested methods.

His-Optimized Cardiac Resynchronization Therapy With Ventricular Fusion Pacing for Electrical Resynchronization in Heart Failure.

OBJECTIVES: This study sought to evaluate the effectiveness of His-optimized cardiac resynchronization therapy (HOT-CRT) for reducing left ventricular activation time (LVAT) compared to His bundle pacing (HBP) and biventricular (BiV) pacing (including multipoint pacing [MPP]), using electrocardiographic (ECG) imaging. BACKGROUND: HBP may correct bundle branch block (BBB) and has shown encouraging results for providing CRT. However, HBP does not correct BBB in all patients and may be combined with univentricular or BiV fusion pacing to deliver HOT-CRT to maximize resynchronization. METHODS: Nineteen patients with a standard indication for CRT, implanted with HBP without correction of BBB and BiV (n = 14) or right ventricular (n = 5) leads, were prospectively enrolled. Patients underwent ECG imaging while pacing in different configurations using different LV electrodes and at different HBP ventricular pacing (VP) delays. The primary endpoint was reduction in LVAT with HOT-CRT, and the secondary endpoints included various other dys-synchrony measurements including right ventricular activation time (RVAT). RESULTS: Compared to HBP, HOT-CRT reduced LVAT by 21% (-17 ms [95% confidence interval [CI]: -25 to -9 ms]; p < 0.001) and outperformed BiV by 24% (-22 ms [95% CI: -33 to -10 ms]; p = 0.002) and MPP by 13% (-11 ms [95% CI: -21 to -1 ms]; p = 0.035). Relative to HBP, HOT-CRT also reduced RVAT by 7% (-5 ms [95% CI: -9 to -1 ms; p = 0.035) in patients with right BBB, whereas RVAT was increased by BiV. The other electrical dyssynchrony measurements also improved with HOT-CRT. CONCLUSIONS: HOT-CRT acutely improves ventricular electrical synchrony beyond BiV and MPP. The impact of this finding needs to be evaluated further in studies with clinical follow-up. (Electrical Resynchronization and Acute Hemodynamic Effects of Direct His Bundle Pacing Compared to Biventricular Pacing; NCT03452462).

Outcome of paediatric portopulmonary hypertension in the modern management era: A case report of 6 patients.

Portopulmonary hypertension is a rare but serious complication of portal hypertension or portosystemic shunting. Portopulmonary hypertension is an indication for liver transplantation or shunt closure. However, liver transplantation is contraindicated in patients with severe pulmonary arterial hypertension. Reported mortality rates are high in children with portopulmonary hypertension and there are scarce recommendations on its management. Our aim was to report on our real-world experience of managing portopulmonary hypertension in a specialised centre. We describe a series of 6 children with portopulmonary hypertension. Their median age at diagnosis was 13 years (range 10-15). The underlying liver conditions were cirrhosis of unknown origin (1), congenital portocaval shunts (3), biliary atresia (1), and portal vein cavernoma with surgical mesenterico-caval shunt (1). Median mean pulmonary arterial pressure was 47 mmHg (range 32-70), and median pulmonary vascular resistance was 6.6 Wood units (range 4.3-15.4). All patients except one were treated with a combination of pulmonary arterial hypertension-specific therapy (phosphodiesterase type 5 inhibitors and/or endothelin receptor antagonists and/or prostacyclin analogues). Three patients then benefited from shunt closure and the others underwent liver transplantation. Five patients showed improvement or stabilisation of pulmonary arterial hypertension with no deaths after a mean follow-up of 39 months. Based on our limited experience, early and aggressive treatment with a combination of pulmonary arterial hypertension-specific therapy significantly improves patients' haemodynamic profile and enables the performance of liver transplantation and shunt closure with satisfactory outcomes.

A 13-Year-Old Male With Diagnosed Idiopathic Pulmonary Hypertension: Is it Really Idiopathic?

CASE PRESENTATION: A 13-year-old male was referred after incidental finding of cardiomegaly on chest radiograph and signs of pulmonary hypertension on subsequent cardiology consult. He was diagnosed with idiopathic pulmonary hypertension, and came to our center for a second opinion. He was born from consanguineous parents. He reported to be asymptomatic in his daily life. He was not on medications. Family history was not contributive.

Unexpected Acceleration in Treprostinil Delivery Administered by a Lenus Pro® Implantable Pump in Two Patients Treated for Pulmonary Arterial Hypertension.

Intravenous treprostinil administration by an implantable pump is an attractive option for pulmonary arterial hypertension (PAH) treatment and is the subject of recent publications. Short-term studies are promising, but there is still a lack of long-term prospective data. We analyzed the treprostinil flow rate administered by the Lenus Pro® implantable pump in 2 patients suffering from PAH during follow-up times of respectively 4.2 and 3 years. The flow rate delivered by the pumps in these 2 patients exceeded the manufacturer admitted margin of error within 2 years and continued to increase to reach, respectively, 158 and 120% of the expected flow rate at the end of the follow up. In one case, the implantable pump had to be removed for this reason. The ex-vivo flow rate of the withdrawn pump determined in the laboratory reached 173% of the predicted value. This correlated with the in-vivo measurement, which suggests a continuous flow increase even after pump removal and without treprostinil use. Spontaneous flow increase from such an implantable pump is a potentially major pitfall, which needs to be identified and actively managed by the responsible clinicians.

A lung graph model for the radiological assessment of chronic thromboembolic pulmonary hypertension in CT.

Chronic thromboembolic pulmonary hypertension (CTEPH) is a possible complication of pulmonary embolism (PE), with poor prognosis if left untreated. Surgical curative treatment is available, particularly in the early stages of the disease. However, most cases are not diagnosed until specific symptoms become evident. A small number of computed tomography (CT) findings, such as a widened pulmonary artery and mosaicism in the lung parenchyma, have been correlated with pulmonary hypertension (PH). Quantitative texture analysis in the CT scans of these patients could provide complementary sub-visual information of the vascular changes taking place in the lungs. For this task, a lung graph model was developed with texture descriptors from 37 CT scans with confirmed CTEPH diagnosis and 48 CT scans from PE patients who did not develop PH. The probability of presenting CTEPH, computed with the graph model, outperformed a convolutional neural network approach using 10 different train/test splits of the data set. An accuracy of 0.76 was obtained with the proposed texture analysis, and was then compared to the visual assessment of CT findings, manually identified by a team of three expert radiologists, commonly associated with pulmonary hypertension. This graph-based score combined with the information attained from the radiological findings resulted in a Cohen's kappa coefficient of 0.47 when differentiating patients with confirmed CTEPH from those with PE who did not develop the disease. The proposed texture quantification could be an objective measurement, complementary to the current analysis of radiologists for the early detection of CTEPH and thus improve patient outcome.

Incidental diagnosis of a silent brachiocephalic vein aneurysm and its surgical treatment.

Brachiocephalic vein aneurysm is an extremely rare clinical entity, with a limited number of case reports in the literature. We report the case of a 74-year-old woman with a left brachiocephalic vein aneurysm (3 × 3 × 6.5 cm) that was discovered fortuitously by contrast-enhanced chest computed tomography. Surgical intervention was performed simultaneously during tricuspid valve surgery with coronary artery bypass grafting. This paper discusses the details of our case, the surgical treatment of brachiocephalic vein aneurysms, and the different treatment options.

"Double-Parachute" Mitral Valve.

A 38-year-old asymptomatic man was referred by his general practitioner for a 3/6 systolic heart murmur, which was detected during a routine consultation. Echocardiography revealed a parachute mitral valve associated with a parachute-like membrane, causing significant subaortic obstruction that was eventually surgically resected with an excellent postoperative outcome. (Level of Difficulty: Beginner.).

Delayed manifestation of COVID-19 presenting as lower extremity multilevel arterial thrombosis: a case report.

BACKGROUND: Venous thrombo-embolic events have been described in hospitalized patients with coronavirus disease 2019 (COVID-19), suggesting the presence of coagulopathy induced by the viral infection. To date, only rare cases of arterial thrombosis related to COVID-19 have been reported. CASE SUMMARY: A 54-year-old patient with an influenza-like illness 15 days earlier, which resolved, and no known cardiovascular risk factor presented with acute right lower limb ischaemia. A computed tomography angiogram of the abdominal aorta and lower extremities showed, in the absence of vascular disease, a subocclusive thrombosis of the right common iliac artery and an occlusion of the right internal iliac, profunda femoral, and popliteal arteries. On the left side, the computed tomography angiogram demonstrated a non-occlusive thrombosis of the common femoral artery. The patient underwent emergency surgical thrombectomy as well as endovascular revascularization on the right side followed by therapeutic anticoagulation, with normalization of the limb perfusion. A nasopharyngeal swab for severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) by real-time reverse transcription-PCR (rRT-PCR) was negative three times. Haemostasis analysis showed a mild hyperfibrinogenaemia and a shortening of the activated partial thromboplastin time. An extensive screening for cardio-embolism was negative. As the thrombotic event was unexplained, antibody testing for SARS-CoV-2 was performed and the result was positive. DISCUSSION: Venous thrombosis and pulmonary embolisms have been observed in COVID-19. As in our case, the first reports on COVID-19-associated arterial thrombotic events have emerged. A better understanding of the coagulopathy in COVID-19 is essential to guide prevention and treatment of venous as well as arterial thrombo-embolic events.

Compressed sensing MRI of different organs: ready for clinical daily practice?

OBJECTIVES: The aim was to evaluate the image quality and sensitivity to artifacts of compressed sensing (CS) acceleration technique, applied to 3D or breath-hold sequences in different clinical applications from brain to knee. METHODS: CS with an acceleration from 30 to 60% and conventional MRI sequences were performed in 10 different applications in 107 patients, leading to 120 comparisons. Readers were blinded to the technique for quantitative (contrast-to-noise ratio or functional measurements for cardiac cine) and qualitative (image quality, artifacts, diagnostic findings, and preference) image analyses. RESULTS: No statistically significant difference in image quality or artifacts was found for each sequence except for the cardiac cine CS for one of both readers and for the wrist 3D proton density (PD)-weighted CS sequence which showed less motion artifacts due to the reduced acquisition time. The contrast-to-noise ratio was lower for the elbow CS sequence but not statistically different in all other applications. Diagnostic findings were similar between conventional and CS sequence for all the comparisons except for four cases where motion artifacts corrupted either the conventional or the CS sequence. CONCLUSIONS: The evaluated CS sequences are ready to be used in clinical daily practice except for the elbow application which requires a lower acceleration. The CS factor should be tuned for each organ and sequence to obtain good image quality. It leads to 30% to 60% acceleration in the applications evaluated in this study which has a significant impact on clinical workflow. KEY POINTS: • Clinical implementation of compressed sensing (CS) reduced scan times of at least 30% with only minor penalty in image quality and no change in diagnostic findings. • The CS acceleration factor has to be tuned separately for each organ and sequence to guarantee similar image quality than conventional acquisition. • At least 30% and up to 60% acceleration is feasible in specific sequences in clinical routine.

An unusual thrombus location in a Heartmate 3TM device with fatal outcome.

Even if the HeartMate 3TM left ventricular assist device is associated with excellent outcomes, complications, such as pump thrombosis continue to affect patients on hemodynamic support. We report the history of a 68-year-old man who underwent implantation of an HeartMate 3TM as a bridge to transplantation. Nineteen months later, he developed signs of heart failure leading to cardiogenic shock. Neither clinical examination nor parameters from the device allowed a clear-cut diagnosis. Only surgical exploration revealed the presence of clots between the polyethylene terephthalate (Dacron®) and polytetrafluoroethylene tubes. This constitutes a weakness of this device for which we propose to the manufacturer for minimal modifications to overcome the problem.

Exclusion of a coronary aneurysm with a polyurethane-covered stent.

A coronary artery aneurysm is a challenging cli-nical situation due to the lack of sufficient evidence from randomized controlled studies and the lack of consensus on a management strategy. The present case is a description of the exclusion of a middle segment aneurysm of the left circumflex coronary artery using a PK Papyrus covered stent (Biotronik, AG, Bulach, Switzerland). The final images were favorable.

Low Iodine Contrast Injection for CT Acquisition Prior to Transcatheter Aortic Valve Replacement: Aorta Assessment and Screening for Coronary Artery Disease.

RATIONALE AND OBJECTIVES: To assess both the complete aorta and coronary artery disease (CAD) using low iodine contrast computed-tomography angiography before transcatheter aortic valve replacement. MATERIALS AND METHODS: 84 patients underwent computed-tomography angiography before transcatheter aortic valve replacement: 42 with standard iodine injection protocol (P1:120 mL); 42 with a low dose iodine injection protocol (P2:60 mL). Mean attenuation and subjective image quality were rated at different levels of the aorta, iliac and coronary arteries. Sensitivity, specificity, negative and positive predictive values for depiction of CAD were calculated according to the coronary angiography. RESULTS: Mean attenuation was significantly higher in P1 for the ascending aorta (p < 0.001). No significant difference was observed regarding image quality of the aortic valve (p = 0.876), the ascending aorta (p = 0.306), or the abdominal aorta (p = 1.0). Diagnostic image quality of coronary arteries was excellent for P1 and P2 (94.6% vs 96.5%, p = 0.08). Sensitivity, specificity, negative and positive predictive values, and accuracy for depiction of CAD were excellent for P1 and P2 (100% vs 100%; 79% vs 86%, 70% vs 87%, 100% vs 100% and 86% vs 93%) without significant differences (p = 0.93; p = 0.58; p = 0.90; p = 1.0; p = 0.74), respectively. CONCLUSION: Despite a difference in aortic mean attenuation, a reduced iodine injection protocol showed similar image quality and detection of CAD in comparison with a standard injection protocol.

Unsuccessful Transfemoral Tricuspid Valve-in-Ring Implantation: Case Report and Literature Review.

Transcatheter tricuspid valve-in-ring implantation has emerged as a potential alternative to surgery for high-risk patients with symptomatic severe tricuspid regurgitation that recurs after surgical ring repair. The worldwide experience remains limited. We report a case of unsuccessful transfemoral tricuspid valve-in-ring implantation (using an Edwards SAPIEN 3 valve, Edwards Lifesciences, Irvine, CA) and literature review. The rigidity, open shape, and open configuration of the ring may lead to imperfect positioning, resulting in severe paravalvular leak. Particular attention should be paid to sizing and wire position with respect to the ring while implanting the valve.

Après annuloplastie tricuspide chirurgicale, l'implantation d'une valve percutanée dans l'anneau représente une potentielle alternative à la reprise chirurgicale chez les patients à haut risque présentant une régurgitation tricuspide sévère symptomatique. L'expérience d'une telle intervention dans le monde demeure toutefois limitée. Nous rapportons un cas d'échec de l'implantation transfémorale d'une valve SAPIEN 3 (Edwards Lifesciences, Irvine, CA) dans un anneau tricuspide et nous passons en revue la littérature. La rigidité ainsi que la forme et la configuration ouverte de l'anneau peuvent conduire à une mauvaise position de la prothèse valvulaire avec comme résultat une fuite paravalvulaire potentiellement sévère. Il faut porter une attention particulière à la sélection de la taille de la prothèse valvulaire et à la position du fil guide au moment de l'implantation de la valve.

3D-printed heart model to guide LAA closure: useful in clinical practice?

OBJECTIVES: Correct device sizing for left atrial appendage (LAA) closure remains challenging due to complex LAA shapes. The aim of our study was to investigative the utility of personalized 3D-printed models (P3DPM) of the LAA to guide device size selection. METHODS: Fifteen patients (75.4 ±8.5years) scheduled for LAA closure using an Amulet device underwent cardiac computed tomography (CT). The LAA was segmented by semiautomatic algorithms using Vitrea® software. A 1.5-mm LAA thick shell was exported in stereolithography format and printed using TangoPlus flexible material. Different Amulet device sizes on the P3DPM were tested. New P3DPM-CT with the device was acquired in order to appreciate the proximal disc sealing the LAA ostium and the compression of the distal lobe within the LAA. We predicted the device size with P3DPM and compared this with the device sizes predicted by transesophageal echocardiography (TEE) and CT as well as the device size implanted in patients. RESULTS: The device size predicted by 3D-TEE and CT corresponded to the implanted device size in 8/15 (53%) and 10/15 (67%), respectively. The predicted device size from the P3DPM was accurate in all patients, obtaining perfect contact with the LAA wall, without device instability or excessive compression. P3DPM-CT with the deployed device showed device deformation and positioning of the disk in relation to the pulmonary veins, allowing us to determine the best device size in all 15 cases. CONCLUSION: P3DPM allowed us to simulate the LAA closure procedure and thus helped to identify the best Amulet size and position within the LAA. KEY POINTS: • A 3D-printed heart model allows to simulate the LAA closure procedure. • A 3D-printed heart model allowed to identify the optimal Amulet size and position. • 3D-printed heart models may contribute to reduce the Amulet implantation learning curve.