ABSTRACT
Takotsubo syndrome is a potentially reversible cause of acute systolic dysfunction. Takotsubo syndrome is rare in children, with no reported dobutamine-induced cases to date. We present a 14-year-old male with prior history of heart transplantation, who developed Takotsubo syndrome during dobutamine stress echocardiography. We highlight the importance of its early recognition to ensure supportive measures with avoidance of inotropic medications.
Subject(s)
Heart Transplantation , Takotsubo Cardiomyopathy , Male , Humans , Child , Adolescent , Dobutamine/adverse effects , Takotsubo Cardiomyopathy/chemically induced , Takotsubo Cardiomyopathy/diagnosis , Echocardiography, Stress/adverse effects , Heart Transplantation/adverse effectsABSTRACT
OBJECTIVES: The clinical implications of finding immobile leaflet(s) at the time of bioprosthetic valve implantation but with acceptable prosthetic haemodynamics are uncertain. We sought to determine the characteristics of such patients and their impact on outcome. METHODS: Patients with immobile leaflet at the time of surgical bioprosthetic valve implantation were identified retrospectively by a systematic search of an institutional echocardiography database (2010-2020). Intraoperative echocardiograms were reviewed de-novo to confirm immobile leaflet(s) at the time of implantation. Cases were matched 1:2 to controls with normal bioprosthetic leaflets motion for age, sex, prosthesis position, prosthesis model, size, year of implantation, and pre-implantation left ventricular ejection fraction. Proportional hazards method was used to analyse the composite endpoint of stroke, valve thrombosis or re-intervention. RESULTS: Immobile leaflet at the time of bioprosthetic valve implantation were found in 26 patients (median age 71 ys 39% males) following tricuspid (n=13), mitral (n=11) and aortic (n=2) valve replacements; 96% received porcine prostheses; prosthesis size was 27 mm or larger in 92%. Immobile leaflet were recorded on intraoperative reports in 16 (62%) cases. It resulted in elevated gradient or mild-moderate prosthetic regurgitation in three (12%), but none led to immediate corrective action intraoperatively. At median follow-up of 21 (4-50) months, presence of immobile leaflet was associated with composite clinical endpoint of stroke, valve thrombosis or re-intervention (hazard ratio 6.8, 95% CI 1.8-25.2, p<0.01) compared to controls. CONCLUSION: Immobile leaflet immediately post-bioprosthetic valve implantation is frequently under-recognised intraoperatively and appears to be associated with early bioprosthetic dysfunction and worse clinical outcome.
Subject(s)
Bioprosthesis , Heart Valve Prosthesis Implantation , Heart Valve Prosthesis , Stroke , Thrombosis , Animals , Bioprosthesis/adverse effects , Female , Heart Valve Prosthesis/adverse effects , Heart Valve Prosthesis Implantation/adverse effects , Heart Valve Prosthesis Implantation/methods , Male , Prosthesis Design , Prosthesis Failure , Retrospective Studies , Risk Factors , Stroke/etiology , Stroke Volume , Swine , Thrombosis/etiology , Treatment Outcome , Ventricular Function, LeftABSTRACT
OBJECTIVES: The purpose of this study was to investigate the influence of simulated reduced-dose three-dimensional angiography (3DA) on the accuracy and precision of linear measurements derived from 3DA datasets. BACKGROUND: Three-dimensional angiography is performed during X-ray guided interventional procedures to aid diagnosis and inform treatment strategies for children and adults with congenital heart disease. However, 3DA contributes substantially to patient radiation dose and may lead to an increased radiation-induced cancer risk. METHODS: Reduced-dose patient 3DA images were simulated by adding quantum noise to the 2D projection angiograms, then reconstructing the projection angiograms into the 3DA dataset. Dose reduction in the range 33-72% was simulated. Five observers performed 46 vessel diameter measurements along prespecified axes within 23 vessel segments from 11 patient 3DA datasets. Statistical tests were performed to assess the influence of radiation dose reduction on the accuracy and precision of vessel diameter measurements. RESULTS: Vessel diameter measurements were in the range 5.9- 22.7 mm. Considering all vessel segments and observers, the influence of dose level on the accuracy of diameter measurements was in the range 0.02 - 0.15 mm (p .05-.8). Interobserver variability increased modestly with vessel diameter, but was not influence by dose level (p = .52). The statistical test for observer recall bias was negative (p = .51). CONCLUSIONS: Simulated dose reduction up to 72% did not affect the accuracy or precision of the diameter measurements acquired from 3DA images. These findings may embolden 3DA radiation dose reduction for pediatric and congenital heart disease patients.
Subject(s)
Cardiology , Drug Tapering , Adult , Angiography, Digital Subtraction , Child , Humans , Imaging, Three-Dimensional , Treatment OutcomeABSTRACT
Antegrade transseptal approach was utilized in the first human case of transcatheter aortic valve replacement (TAVR) and in the early phase of TAVR. Various challenges with the antegrade transseptal approach including procedural complexity, need for atrial septal crossing, and potential for injury to the mitral valve apparatus led it to being supplanted by other approaches. These challenges have now largely been mitigated as structural interventionalists routinely perform left atrial procedures. We report a case of antegrade transseptal TAVR using a large bore sheath placed in the mid left ventricle across the mitral orifice to protect the mitral valve apparatus and facilitate valve deployment. An 84-year-old man with heart failure symptoms was seen for severe aortic stenosis. The severity of peripheral arterial disease precluded femoral, axillary, carotid, or transcaval routes. After transseptal puncture and creation of an arteriovenous rail, a long 26-Fr sheath was advanced from the right femoral vein transseptally over the arteriovenous rail, past the mitral valve inflow to the mid left ventricular cavity. The sheath provided a stable platform with stable intraprocedure hemodynamics. Balloon valvuloplasty was performed in an antegrade manner, after which a 29-mm SAPIEN S3 prosthesis was advanced into the aortic valve position and deployed under rapid pacing. We observed no injury to the mitral leaflets or subvalvular apparatus after the procedure. The antegrade transseptal approach should be revisited as an option for patients at high surgical risk with no other suitable access site. The use of a large bore sheath facilitates this approach.
Subject(s)
Aortic Valve Stenosis/surgery , Aortic Valve/surgery , Cardiac Catheterization , Transcatheter Aortic Valve Replacement , Aged, 80 and over , Aortic Valve/diagnostic imaging , Aortic Valve/physiopathology , Aortic Valve Stenosis/diagnostic imaging , Aortic Valve Stenosis/physiopathology , Cardiac Catheterization/instrumentation , Cardiac Catheters , Fatal Outcome , Heart Failure/diagnostic imaging , Heart Failure/physiopathology , Heart Valve Prosthesis , Humans , Male , Severity of Illness Index , Transcatheter Aortic Valve Replacement/instrumentation , Treatment OutcomeABSTRACT
OBJECTIVE: Perforate the expanded polytetrafluoroethylene membrane of the GORE® CARDIOFORM Septal Occluder (GSO) and GORE® CARDIOFORM ASD Occluder (GCA) after implantation. BACKGROUND: Percutaneous transseptal access to the left atrium is necessary for many structural and electrophysiological procedures. The potential need to access the left atrium may influence decision-making for patent foramen ovale or atrial septal defect closure. METHODS: Sixteen canines underwent implantation of equal number GSO or GCA devices. A transseptal crossing procedure was performed through the device 85 (±1) days postoccluder implantation. The crossing procedure was performed utilizing commercially available equipment: radiofrequency/SureFlex sheath and standard needle/Mullin's sheath. Progressive dilation of the perforation was performed to allow passage of a 12 French Mullin's sheath into the left atrium. RESULTS: Left atrial access was achieved in all cases. Postmortem analysis demonstrated passage through both occluder discs in all radiofrequency/SureFlex sheath cases (4 GSO, 4 GCA) and half of the standard needle/Mullin's sheath cases (3 GSO, 1 GCA). The remaining standard needle/Mullin's sheath cases demonstrated perforation through the right atrial disc but passage around the septal aspect of the left atrial disc, thus not perforating the left atrial disc. No acute embolic complications from the procedure were observed. CONCLUSIONS: Left atrial access may be achieved through the GSO or GCA devices after implantation and endothelialization. The combination of a radiofrequency needle and steerable sheath provides benefit over a standard needle and Mullin's sheath in accomplishing passage through both occluder discs.
Subject(s)
Cardiac Catheterization/instrumentation , Cardiac Catheters , Catheter Ablation/instrumentation , Heart Septum , Septal Occluder Device , Animals , Dogs , Female , Heart Septum/diagnostic imaging , Male , Polytetrafluoroethylene , Prosthesis Design , Punctures , Time FactorsABSTRACT
A healthy 20-year-old male presented with aborted sudden death. Both thoracic echocardiography and cardiac computed tomographic angiography demonstrated the characteristic findings of ALCAPA noting a dilated and tortuous right coronary artery, dilated coronary collateral arteries within the interventricular septum or along the surface of the heart, and visualization of the left coronary artery origin from the posterior aspect of the main pulmonary artery. Both imaging modalities demonstrated all three characteristic findings of this rare coronary anomaly and served as complementary imaging studies for surgical correction.
Subject(s)
Bland White Garland Syndrome/diagnosis , Computed Tomography Angiography/methods , Coronary Angiography/methods , Coronary Vessels/diagnostic imaging , Echocardiography, Doppler, Color/methods , Pulmonary Artery/diagnostic imaging , Ventricular Septum/diagnostic imaging , Electrocardiography , Humans , Imaging, Three-Dimensional , Male , Pulmonary Artery/abnormalities , Young AdultABSTRACT
PURPOSE OF REVIEW: Patients with complete and congenitally corrected transposition of the great arteries commonly survive into adulthood and present with a vast array of clinical residua. RECENT FINDINGS: Echocardiography remains the primary imaging modality in the routine assessment of the adult with transposition of the great arteries. It provides a comprehensive anatomic and hemodynamic evaluation. Limitations to echocardiography include evaluation of the following: the systemic right ventricle, baffle patency following atrial switch procedure, coronary arteries following arterial switch procedure or Nikadoh, and multilevel right ventricular outflow tract obstruction. SUMMARY: Each form of palliation for transposition of the great arteries results in unique long-term sequelae that affect outcomes. A multimodality approach to imaging is required for a complete evaluation.
Subject(s)
Arteries/diagnostic imaging , Echocardiography , Transposition of Great Vessels/diagnostic imaging , Adult , Heart Ventricles , HumansABSTRACT
OBJECTIVES: To develop a novel delivery technique to overcome vascular size limitations for device closure of a secundum ASD in the young patient. BACKGROUND: The efficacy, clinical utility, and safety of transcatheter closure in comparison to surgical closure are well established; however, access vessel size remains a potential limitation to device selection in the young patient. METHODS: A retrospective medical record review of all cases of secundum ASD closure between April 2015 and April 2016 utilizing a novel delivery method described herein at two separate academic institutions. The device is disassembled allowing the delivery sheath to serve as the introducer. It is advanced to the RA with re-introduction of the device allowing for deployment in the standard fashion. RESULTS: Overall, 10 patients underwent secundum ASD closure via this novel delivery technique. There were six females (60%) and the average age at time of the procedure was 4.2 years ± 1.6 years (range 2-6 years). The majority of patients had an isolated secundum ASD (70%) with the primary indication for closure being right ventricular volume overload (90%). All patients had successful placement of a GORE® CARDIOFORM® Septal Occluder (GCSO) with no or trivial residual shunt. No patients had vascular complications related to the procedure. CONCLUSIONS: We describe a novel technique for ASD closure using the GCSO delivery sheath as the access sheath, which reduces the vascular access size requirement by 25%, thus addressing one common limitation for percutaneous device closure of a secundum ASD in young patients. © 2016 Wiley Periodicals, Inc.
Subject(s)
Cardiac Catheterization/instrumentation , Cardiac Catheterization/methods , Heart Septal Defects, Atrial/therapy , Septal Occluder Device , Age Factors , Cardiac Catheterization/adverse effects , Child , Child, Preschool , Female , Heart Septal Defects, Atrial/diagnostic imaging , Heart Septal Defects, Atrial/physiopathology , Humans , Male , Medical Records , Minnesota , Prosthesis Design , Retrospective Studies , Risk Factors , San Francisco , Time Factors , Treatment OutcomeABSTRACT
Over the last 15 years, stent placement has become a viable and safe alternative to surgical repair for native and recurrent coarctation of the aorta. Although patients with coarctation of the aorta often have lower exercise tolerance, there is no published data regarding the effect of coarctation stenting on exercise capacity. We aimed to determine the effect of coarctation stent placement on exercise capacity. We conducted a retrospective chart review of all patients who underwent stent placement for native or recurrent coarctation of the aorta at our institution from January 2000 to October 2012. We identified those patients who underwent exercise testing before and after stent placement. We compared measures of exercise capacity pre- and post-stent placement using paired t tests. Among 48 patients who underwent coarctation stenting, 7 (mean age = 40, range 21-65 years) were found to have isolated coarctation of the aorta and had the necessary pre- and post-stent exercise data. Only one patient had native coarctation of the aorta. The peak-to-peak systolic gradient improved by a mean of 26 mmHg (p < 0.02) after stent placement. Functional aerobic capacity (FAC) improved from a mean of 69-83% after stent placement (p < 0.03). Metabolic equivalents (METs) also improved significantly from a mean of 8.7 to 10.2 (p < 0.03). In our cohort, stent placement for relief of recurrent coarctation of the aorta resulted in a significant improvement in exercise capacity. This improvement may not be as significant in patients with milder coarctation of the aorta or those with complicating morbidities. Larger studies with more long-term follow-up are needed to better quantify this improvement and to define any lasting effect of this intervention on exercise tolerance.
Subject(s)
Aortic Coarctation/physiopathology , Aortic Coarctation/surgery , Exercise Tolerance , Adult , Aged , Blood Vessel Prosthesis Implantation , Exercise Test , Female , Humans , Male , Middle Aged , Retrospective Studies , Stents , Young AdultABSTRACT
SVC obstruction is a rare acute complication after bicaval cardiac transplantation and can be catastrophic for the patient. Herein, we report the case of a child who developed SVC thrombosis causing acute SVC syndrome nine days after heart transplantation. Thrombolysis was successfully performed using the AngioJet™ system and subsequent balloon angioplasty with re-establishment of flow. This procedure allowed for revascularization without repeat sternotomy or stent implantation. The patient is well without recurrence at last follow-up.
ABSTRACT
We present a 19-year-old female with history of d-transposition of the great arteries status post-arterial switch operation.
Subject(s)
Transposition of Great Vessels , Female , Humans , Young Adult , Cardiac Catheters , Pulmonary Artery/surgery , Stents/adverse effects , Transposition of Great Vessels/diagnosis , Transposition of Great Vessels/surgery , Treatment OutcomeABSTRACT
A 24-year-old female with history of an atrial septal defect post-patch closure (bovine pericardium) presented 4 years postoperative with an incidentally identified mass originating from the septal patch .
Subject(s)
Heart Septal Defects, Atrial , Neoplasms , Female , Humans , Young Adult , Heart Septal Defects, Atrial/surgery , Neoplasms/diagnosis , Suction , Treatment OutcomeABSTRACT
BACKGROUND: Pulmonary atresia with ventricular septal defect without major aortopulmonary collateral arteries (MAPCAs) is an uncommon form of congenital heart disease. As more patients with congenital heart disease live to adulthood, the objective of this article was to review the long-term results of this specific population. METHODS: A review of the PubMed database was performed using pertinent key words (pulmonary atresia, tetralogy of Fallot, conduit, right ventricle-pulmonary artery) concentrating on studies from 1990-present and published in English. Most studies of pulmonary atresia-ventricular septal defect patients included those with and without MAPCAs. Analysis included examination of the entire cohort, consideration of the proportion of patients with MAPCAs, and any subgroup analysis of the patients without MAPCAs. RESULTS: Survival is approximately 80% at ten years and is improved with complete repair and larger pulmonary arteries. Some studies have found genetic syndromes and extracardiac anomalies to impact survival, while others have not. Incomplete repair has been shown to be associated with worse survival. Independent of initial management strategy, patients with pulmonary atresia and ventricular septal defects without MAPCAs require repeat intervention on the right ventricular outflow tract. Hypoplastic pulmonary arteries have been shown to be a risk factor for reintervention, and decreased conduit durability has been shown with younger age at implantation of conduit. CONCLUSIONS: Long-term outcomes have improved for patients with pulmonary atresia-ventricular septal defect without MAPCAs, with complete repair and adequate pulmonary arteries favorable for survival. Long-term outcomes include reinterventions, both catheter-based and surgical, predominantly on the right ventricular outflow tract.
ABSTRACT
BACKGROUND: There are limited data about changes in cardiac function (cardiac reverse remodeling) and heart failure indices after transcatheter tricuspid valve-in-valve replacement (TT-VIVR). The purpose of this study was to evaluate cardiac reverse remodeling and temporal changes in heart failure indices after TT-VIVR in adults with congenital heart disease. METHODS: Retrospective cohort study of adults with congenital heart disease that underwent TT-VIVR and had >6 months of follow-up (January 1, 2011, to April 30, 2023). Echocardiographic indices of cardiac remodeling and heart failure indices (New York Heart Association class, NT-proBNP (N-terminal pro-brain natriuretic peptide), glomerular filtration rate, and model for end-stage liver disease excluding international normalized ratio score) were assessed preintervention and at 1-, 3-, and 5-year postintervention. RESULTS: Of 39 patients (age 39 [32-46] years), 14 (36%) and 25 (64%) received Melody valve and Sapien valve prosthesis, respectively. At 1-year post-TT-VIVR, there was a temporal improvement in right atrial reservoir strain (17±8% versus 22±8%, P<0.001), right atrial volume (81 [59-108] versus 63 [48-82] mL/m2, P<0.001), right atrial pressure (12±4% versus 6±4%, P<0.001), and right ventricular global longitudinal strain (-15±7% versus -20±7%, P<0.001). Similarly, there was a temporal improvement in NT-proBNP, glomerular filtration rate, model for end-stage liver disease excluding international normalized ratio score, and New York Heart Association class. The temporal improvements in heart failure indices and valve function were maintained at 3- and 5-year post-TT-VIVR. CONCLUSIONS: Considering the significant mortality risk associated with reoperations for tricuspid valve replacement, these data suggest favorable outcomes after TT-VIVR, and support TT-VIVR as a viable alternative to surgical tricuspid valve replacement, especially in high-risk patients.
Subject(s)
End Stage Liver Disease , Heart Defects, Congenital , Heart Failure , Heart Valve Prosthesis Implantation , Tricuspid Valve Insufficiency , Adult , Humans , Tricuspid Valve/diagnostic imaging , Tricuspid Valve/surgery , End Stage Liver Disease/etiology , End Stage Liver Disease/surgery , Heart Valve Prosthesis Implantation/adverse effects , Retrospective Studies , Treatment Outcome , Severity of Illness Index , Tricuspid Valve Insufficiency/diagnostic imaging , Tricuspid Valve Insufficiency/surgery , Heart Defects, Congenital/diagnostic imaging , Heart Defects, Congenital/surgery , Heart Failure/diagnostic imaging , Heart Failure/therapy , Heart Failure/etiologyABSTRACT
The purpose of this study was to evaluate left ventricular (LV) unloading strategies in patients supported with peripheral venoarterial extracorporeal membrane oxygenation (VA-ECMO). A retrospective review was conducted of all consecutive patients requiring VA-ECMO support for any indication, who underwent novel LV unloading strategies with either direct left atrial venoarterial (LAVA) cannulation or pulmonary artery venoarterial (PAVA) venting, in comparison to Impella and intra-aortic balloon pump (IABP). The primary outcome was successful bridge to transplant, LV assist device, or myocardial recovery. Forty-six patients (63% male, mean age 52.8 ± 17.6 years) were included. Fourteen patients (30%) underwent novel unloading with either LAVA or PAVA, 11 patients (24%) underwent IABP placement, and 21 patients (46%) underwent Impella insertion. In the novel LV unloading cohort, 10 patients (71%) survived to hospital discharge. Four patients (29%) were weaned from ECMO and eight patients (57%) underwent cardiac transplantation. Although a trend favoring cannula-based unloading for the primary outcome was noted, the cohort was too small for statistical significance (79% LAVA/PAVA, 57% Impella, 45% IABP; p = 0.21). However, probability of survival was greater in the LAVA/PAVA cohort compared to Impella and IABP ( p < 0.05). Thus, we demonstrate the efficacy of LA and PA cannulation as an alternative LV unloading strategy for patients supported with peripheral VA-ECMO.
Subject(s)
Extracorporeal Membrane Oxygenation , Heart-Assist Devices , Intra-Aortic Balloon Pumping , Humans , Extracorporeal Membrane Oxygenation/methods , Male , Middle Aged , Female , Retrospective Studies , Adult , Aged , Intra-Aortic Balloon Pumping/methods , Treatment Outcome , Heart Failure/therapy , Heart Failure/physiopathology , Heart Failure/surgeryABSTRACT
BACKGROUND: There are limited data about postprocedural right heart reverse remodeling and long-term prosthesis durability after transcatheter pulmonary valve replacement (TPVR) and how these compare to surgical pulmonary valve replacement (SPVR). OBJECTIVES: This study sought to compare right heart reverse remodeling, pulmonary valve gradients, and prosthetic valve dysfunction after TPVR vs SPVR. METHODS: Patients with TPVR were matched 1:2 to patients with SPVR based on age, sex, body surface area, congenital heart lesion, and procedure year. Right heart indexes (right atrial [RA] reservoir strain, RA volume index, RA pressure, right ventricular [RV] global longitudinal strain, RV end-diastolic area, and RV systolic pressure) were assessed at baseline (preintervention), 1 year postintervention, and 3 years postintervention. Pulmonary valve gradients were assessed at 1, 3, 5, 7, and 9 years postintervention. RESULTS: There were 64 and 128 patients in the TPVR and SPVR groups, respectively. Among patients with TPVR, 46 (72%) and 18 (28%) received Melody (Medtronic) vs SAPIEN (Edwards Lifesciences) valves, respectively. The TPVR group had greater postprocedural improvement in RA reservoir strain and RV global longitudinal strain at 1 and 3 years. The TPVR group had a higher risk of prosthetic valve dysfunction mostly because of a higher incidence of prosthetic valve endocarditis compared to SPVR but a similar risk of pulmonary valve reintervention because some of the patients with endocarditis received medical therapy only. Both groups had similar pulmonary valve mean gradients at 9 years postintervention. CONCLUSIONS: These data suggest a more favorable right heart outcome after TPVR. However, the risk of prosthetic valve endocarditis and prosthetic valve dysfunction remains a major concern.
Subject(s)
Endocarditis, Bacterial , Endocarditis , Heart Valve Prosthesis , Pulmonary Valve , Humans , Pulmonary Valve/diagnostic imaging , Pulmonary Valve/surgery , Treatment OutcomeABSTRACT
A 32-year-old man with history of hypoplastic left heart syndrome status post-Fontan palliation (20-mm aortic homograft conduit) had previously undergone Fontan conduit (FC) and left pulmonary artery (LPA) stenting to relieve conduit obstruction.
Subject(s)
Fontan Procedure , Hypoplastic Left Heart Syndrome , Thrombosis , Male , Humans , Adult , Stents , Aorta , Fontan Procedure/adverse effects , Hypoplastic Left Heart Syndrome/diagnosis , Hypoplastic Left Heart Syndrome/surgery , Pulmonary Artery/diagnostic imaging , Pulmonary Artery/surgery , Thrombosis/diagnosis , Thrombosis/etiology , Thrombosis/surgeryABSTRACT
BACKGROUND: There are conflicting data about the association between obesity and clinical outcomes in adults with congenital heart disease (CHD), and the effects of weight gain or weight loss remain unclear. The purpose of this study was to determine whether a temporal change in body mass index (BMI) was associated with clinical outcomes independent of baseline BMI in adults with CHD. METHODS: Retrospective cohort study of adults with CHD that had clinical assessments at baseline and 5 years afterwards. Weight gain and weight loss were defined as ≥5% change from baseline BMI. Atherosclerotic cardiovascular disease (ASCVD) risk profile (blood pressure [BP], low density lipoprotein cholesterol [LDL-C] and hemoglobin A1C [HBA1c]) and cardiovascular events (heart failure hospitalization, transplant, death) were ascertained. RESULTS: Of the 3407, 1804 (53%) had stable weight, 1291 (38%) had weight gain, and 312 (9%) had weight loss at follow-up assessment. The median change in BMI (∆ BMI) was +3% (1-5). The baseline BMI and ∆ BMI were associated with worsening ASCVD risk profile (higher BP, LDL-C and HBA1C), and an increase in cardiovascular events in patients with obesity at baseline. CONCLUSIONS: Collectively, the results provide evidence to support lifestyle interventions aimed at weight maintenance in patients with normal weight, and to promote weight loss in patients that are overweight or obese. Further studies are required to determine the optimal type of lifestyle interventions that will be most effective in this population.
Subject(s)
Atherosclerosis , Heart Defects, Congenital , Adult , Humans , Cholesterol, LDL , Prognosis , Retrospective Studies , Weight Loss , Overweight/epidemiology , Obesity/diagnosis , Obesity/epidemiology , Body Mass Index , Weight Gain , Atherosclerosis/complications , Heart Defects, Congenital/diagnosis , Heart Defects, Congenital/epidemiology , Heart Defects, Congenital/complications , Risk FactorsABSTRACT
A 7-year-old child with native coarctation of the aorta was treated by transcatheter therapy using a 29-mm balloon-expandable stent. The procedure was successful and free of complications, and the patient was discharged home the same day. This stent has several features making it uniquely advantageous for treating this condition. (Level of Difficulty: Advanced.).
ABSTRACT
AIMS: Despite their universal predisposition, diagnosing diastolic dysfunction in patients post-Fontan palliation is challenging. Our aim was to compare exercise haemodynamics between adults post-Fontan and patients with heart failure with preserved ejection fraction (HFpEF) and non-cardiac dyspnoea (NCD). METHODS AND RESULTS: Twenty-four adults (age ≥18 years) post-Fontan palliation with resting and exercise pulmonary artery wedge pressure (PAWP) measured during supine biking were identified. Forty-eight patients with HFpEF and 48 with NCD diagnosed at catheterization were selected for comparison. Mean age for Fontan patients was 30.3 ± 7.5 years; median ventricular ejection fraction was 52.5% (45-55.8), being <50% in 37.5%. Resting PAWP among Fontan patients was 10.2 ± 3.5 mmHg (>12 mmHg in 25%); PAWP was lower in Fontan patients than in HFpEF but higher than NCD. During exercise, PAWP was lower in the Fontan group than HFpEF (22.5 mmHg [19.3-28] vs. 28.2 ± 6.3; p = 0.0006) but higher than NCD (11.2 ± 4.2, p ≤ 0.0001). However, there were no differences in ΔPAWP/ΔQs between Fontan and HFpEF patients (4.0 [2.1-7.3] vs. 2.7 [1.6-4.4]; p = 0.10) with the ratio being higher post-Fontan than in NCD (0.6 [0.2-1.2]; p < 0.0001). ΔPAWP/ΔQs remained similar between HFpEF and Fontan patients even when those with ejection fraction <50% were excluded (2.7 [1.6-4.4] vs. 2.7 [1.0-5.8]; p = 0.97). CONCLUSION: There were no differences in ΔPAWP/ΔQs ratios between post-Fontan and HFpEF patients, supporting markedly abnormal single ventricle compliance despite lower resting and exercise PAWP. Therefore, exercise invasive haemodynamics may represent a novel tool for the diagnosis of diastolic dysfunction in Fontan patients.