Introduction: Psychiatric and cognitive deficiencies are increasingly appreciated in pulmonary arterial hypertension (PAH). Brain abnormalities potentially underlying these disease manifestations have been ill-defined. Hypothesis: Altered brain structure and functional connectivity correlate with neurocognitive changes in PAH patients. Methods: PAH patients at UPMC were enrolled from 2017 to 2018. Participants underwent T1/T2 structural and resting functional brain MRI (fMRI) in addition to cognitive testing. Case data were compared in a 1:4 ratio to that of age-, gender-, and education-matched non-PAH controls. Linear regression analysis with clustered sandwich estimator and adjustment for multiple hypothesis testing was used for comparisons. Most recent hemodynamic data for PAH patients were reviewed including pulmonary vascular resistance (PVR). Pearson correlations were computed between PVR and brain volumes. Results: 12 PAH patients (median age, 45 years [range 26-61]; 9 women) were enrolled. T1/T2 MRI analysis identified 18 regions with volumetric differences (q<0.05), with notable involvement of limbic (6/18, 33%) and basal ganglia (4/18, 22%) structures. PAH patients showed decreased left cerebellar cortical (45178 ± 1417 vs 53295 ± 583 mm 3 ; q=0.01) and right cerebellar white matter volumes (16458 ± 727 vs 39675 ± 5846 mm 3 ; q=0.018). Structural changes concorded with results from fMRI analysis: PAH patients had decreased functional connectivity in the left cerebellum (q=0.003). Corresponding with modulation of executive functions by the cerebellum, a trend was seen toward decreased inhibitory control and attention (83 ± 3 vs 95 ± 1; q=0.07) and pattern comparison processing speed (93 ± 5 vs 106 ± 4; q=0.07) in PAH patients. A trend toward negative correlation of PVR with left cerebellar white matter volume (r = -0.82, q = 0.06) was also observed. Conclusion: Limbic, basal ganglia, and cerebellar regions were smaller and less functionally connected in PAH cases than in matched controls. Decreased cerebellar volume may correlate with more severe hemodynamic disease. Any links among brain changes, cognitive dysfunction, and PAH pathophysiology deserve further study and may lead to neurologic modalities to diagnose and treat disease.

Holodiastolic flow reversal in the descending aorta on echocardiogram suggests significant aortic regurgitation. The study aim was to determine whether the presence of holodiastolic flow reversal on cardiac magnetic resonance imaging (MRI) correlates with aortic valve regurgitant fraction. We retrospectively reviewed 166 cardiac MRIs (64 % male, age 14.1 ± 9.5 years) from January 2011 to May 2012 where velocity mapping was acquired at both the aortic valve and the descending aorta at the level of the diaphragm. Descending aorta velocity maps were checked for baseline offset using a static reference region. Holodiastolic flow reversal was defined as flow reversal throughout diastole both before and after baseline correction. Significant aortic regurgitation was defined as regurgitant fraction >10 %. Aortic valve regurgitant fraction was <10 % in 144 patients (Group A), 10–20 % inclusive in 7 patients (Group B), and >20 % in 15 patients (Group C). Though the aortic valve regurgitant fraction was significantly higher for patients with holodiastolic flow reversal versus those without (8.5 ± 14.2 vs. 3.8 ± 6.6 %, p = 0.02), holodiastolic flow reversal was present in 32 Group A patients (22 %). In comparison, 4 Group B patients (57 %) and 7 Group C patients (47 %) had holodiastolic flow reversal. The sensitivity (Groups B and C) was 0.5, and the specificity (Group A) was 0.78. Holodiastolic flow reversal in the descending aorta on cardiac MRI was neither sensitive nor specific for predicting significant aortic regurgitation in this study population. Holodiastolic flow reversal in the absence of significant aortic regurgitation may be a relatively common finding in patients with congenital heart disease.

Objectives: Using cardiac magnetic resonance imaging (MRI) with virtual angioscopy, we sought to evaluate coronary anatomy, myocardial injury, and left ventricular function in children with interarterial anomalous aortic origin of coronary artery before and after surgery.Methods: We prospectively enrolled children 5 to 19 years old with interarterial anomalous coronary artery. Cardiac MRI was performed with respiratory-navigated steady-state free-precession 3-dimensional data set acquisition. Virtual angioscopy was used to evaluate the coronary ostia directly. Surgery consisted of the modified unroofing procedure.Results: We enrolled 9 subjects between February 2009 and May 2015. Subjects were male, with an average age at surgery of 14.1 years. Anomalous coronary was right in 7 patients (77%) and left in 2. In all subjects, the proximal anomalous coronary arose tangential to the aorta with an elliptical, slitlike ostium. Before the operation, the proximal coronary artery was significantly smaller proximally than distally (2 vs 3.1 mm; P < .0001. After the operation, neo-orifices were round and patent in 7 subjects; however, 2 subjects still had narrowed neo-orifices. New postoperative MRI findings included flattened septal wall motion (N = 1), small region of midmyocardial partial-thickness scar (N = 1), and dyskinetic septal wall motion with mild aortic insufficiency (N = 1). Left ventricular function was normal both before and after surgery (P = .85).Conclusions: Cardiac MRI with virtual angioscopy is an important tool for evaluating anomalous coronary anatomy, myocardial function, and ischemia and should be considered for initial and postoperative assessment of children with anomalous coronary arteries.

Introduction Conotruncal Anomalies patients may require branch pulmonary arterial (BPA) stent implantation. These ferromagnetic stents generate localized artifact that may affect phase contrast magnetic resonance (PCMR) imaging. There is little data on PCMR measurements in the presence of a BPA stent. Hypothesis PCMR acquired measurements away from the BPA stent artifact are accurate and internally consistent. Methods We retrospectively reviewed 43 consecutive repaired Conotruncal Anomalies PCMR and cine MR studies between 2005 and 2012. All patients underwent either left (30) pulmonary artery (LPA) or right (20) pulmonary artery (RPA) stent placement. Three different methods of measuring flow through the stented artery were compared: 1) main pulmonary artery (MPA) - unstented artery, 2) direct PCMR of the stented artery away from the artifact, and 3) pulmonary venous (PV) flows (see Figure). Where available, pulmonary radionuclide scintigraphy (PRS) was compared to each method. Significance was P < 0.05. Results For the entire study population, the mean age and body surface area were 12.0 ± 7.0 yrs (range 1.5 - 33.2 yrs) and 1.2 ± 0.5 m2. There was good agreement without significant difference between the mean PRS and MPA derived stented artery flow (method 1) (37 ± 17 and 35 ± 21 %, r=0.731, ICC=0.8403, p=0.627) There was even better agreement between PRS and direct measurement (method 2) (39 ± 20 and 40 ± 22%, r=0.888, ICC = 0.943, p=0.757). The greatest agreement was found between method 2 and PV flows (method 3) (48 ± 17 and 47 ± 14%, , r=0.939, ICC=0.961, p=0.837). As another check for internal consistency, there was excellent correlation between the PCMR and cine MR right ventricular stroke volume 70.2 ± 40.4 and 74.0 ± 41.4 cc, r=0.983, ICC=0.989. Conclusion In the presence of a ferromagnetic BPA stent, accurate measurement of the net fractional flow ratio is feasible. PCMR adjacent to the stent artifact or pulmonary venous flows provide the most internally consistent data.

Background— Pulmonary insufficiency is the nexus of late morbidity and mortality after transannular patch repair of tetralogy of Fallot. This study aimed to establish the feasibility of implantation of the novel Medtronic Harmony transcatheter pulmonary valve (hTPV) and to assess its effect on pulmonary insufficiency and ventricular function in an ovine model of chronic postoperative pulmonary insufficiency. Methods and Results— Thirteen sheep underwent baseline cardiac magnetic resonance imaging, surgical pulmonary valvectomy, and transannular patch repair. One month after transannular patch repair, the hTPV was implanted, followed by serial magnetic resonance imaging and computed tomography imaging at 1, 5, and 8 month(s). hTPV implantation was successful in 11 animals (85%). There were 2 procedural deaths related to ventricular fibrillation. Seven animals survived the entire follow-up protocol, 5 with functioning hTPV devices. Two animals had occlusion of hTPV with aneurysm of main pulmonary artery. A strong decline in pulmonary regurgitant fraction was observed after hTPV implantation (40.5% versus 8.3%; P =0.011). Right ventricular end diastolic volume increased by 49.4% after transannular patch repair (62.3–93.1 mL/m 2 ; P =0.028) but was reversed to baseline values after hTPV implantation (to 65.1 mL/m 2 at 8 months, P =0.045). Both right ventricular ejection fraction and left ventricular ejection fraction were preserved after hTPV implantation. Conclusions— hTPV implantation is feasible, significantly reduces pulmonary regurgitant fraction, facilitates right ventricular volume improvements, and preserves biventricular function in an ovine model of chronic pulmonary insufficiency. This percutaneous strategy could potentially offer an alternative for standard surgical pulmonary valve replacement in dilated right ventricular outflow tracts, permitting lower risk, nonsurgical pulmonary valve replacement in previously prohibitive anatomies.

There is an established association between tetralogy of Fallot and partial anomalous pulmonary venous connections. This association is important because surgically repaired tetralogy patients have increased risk of right heart failure. We hypothesize that partial anomalous venous connections increase right ventricular volumes and worsen right ventricular failure.

Introduction: Holodiastolic flow reversal (HDR) measured at the descending aorta (DAO) has been used as a surrogate marker to identify significant aortic regurgitation using echocardiography. Hypothesis: The purpose of this study is to determine if the presence of HDR correlates with the aortic valve regurgitant fraction (AVRF). Methods: We retrospectively reviewed 167 CMR studies (64% male, 36% female) from January 2011 to May 2012 where velocity mapping was acquired at both the aortic valve and the DAO at the level of the diaphragm. Patients with coarctation of the aorta or single ventricle physiology were excluded from the study. Descending aortic velocity maps were checked for baseline offset using a static reference region. HDR was defined as flow reversal throughout diastole both before and after baseline correction. Significant aortic regurgitation was defined as an AVRF >10%. Results: There were 145 patients (mean patient age was 14.1 ± 9.5 yrs) with an AVRF 20% (Group C) (Figure 1). Though the AVRF was significantly higher for HDR versus non-HDR pts (8.5 ± 14.2 vs 3.8 ± 6.6%, p=0.04), HDR was present in 32 Group A pts (22%). In comparison, 4 Group B pts (57%), and 7 Group C pts (47%) had HDR. Of the 64 Group A pts with either Tetralogy of Fallot (48) or Transposition (16), 15 pts (23%) had HDR. The sensitivity of HDR to predict the combined Groups B & C was 0.5, and the specificity for Group A was 0.78. Conclusions: DAO HDR is neither a very sensitive nor specific finding for predicting significant aortic regurgitation. HDR in the absence of significant aortic regurgitation appears to be a relatively common finding, especially in patients with repaired conotruncal anomalies. HDR should be interpreted with caution when evaluating aortic insufficiency, and likely has no role in the pediatric and young adult population or in patients after repair of conotruncal anomalies. ![][1] [1]: /embed/graphic-1.gif

Background: Anomalous aortic origin of a coronary artery (AAOCA) with an interarterial course is associated with sudden cardiac death in children. Objectives: Using cardiac MRI with adenosine, we evaluated coronary ostial stenosis, proximal coronary size, and left ventricular (LV) function in children with AAOCA. Methods: We prospectively enrolled children 5-18 years old with interarterial AAOCA. MRIs were reviewed for coronary artery origin, proximal course, dimensions, and cardiac function. Surgery consisted of the modified unroofing procedure. We used descriptive statistics and paired t-tests to evaluate for statistical significance. Results: Between 2/2009 and 5/2014, 24 subjects with AAOCA underwent 29 MRIs. The majority were male (N=19, 79%) with anomalous right coronary artery (AAORCA, N=20, 83%). Mean age was 12.8 years at time of initial MRI. MRI was performed an average of 7 months post-operatively in 8 subjects who underwent surgery. In all, the proximal anomalous coronary arose tangential to the aorta with an elliptical, slit-like ostium. The anomalous coronary measured smaller proximally (0.20 mm) compared to distally (0.31 mm, P=< 0.0001), and after surgical repair, the post-operative origin was significantly larger (0.36 vs. 0.21 mm, P=0.02). Other abnormalities at initial MRI included fixed inferior wall (N=1) and reversible subendocardial septal/inferior wall (N=1) perfusion defects. Post-operatively, the neo-ostium was round in 6 (see Figure), but in 2, the orifice remained elliptical. One patient had a new small mid-myocardial scar and one had dyskinetic septal wall motion. LV function was normal both before and after surgery (mean ejection fraction =68.1% vs. 67.5%, P=0.85). Conclusions: Cardiac MRI with adenosine is an important tool for the evaluation of anomalous anatomy, myocardial function, and ischemia/injury and should be considered for the initial and, when applicable, post-operative assessment of children with AAOCA.