Weight loss in hypertrophic cardiomyopathy: A clinical case series.

Background: Obesity is prevalent among patients with hypertrophic cardiomyopathy (HCM). Obese HCM patients have greater wall thickness, LV mass, worse hemodynamic function and NYHA class. Weight loss may favorably influence the HCM phenotype. Case summary: We describe six patients with hypertrophic cardiomyopathy who lost weight through diet and lifestyle changes (n = 4) or bariatric surgery (n = 2). Radiographic imaging, with cardiac MRI or CT scan, was performed before and after their weight loss. There was a mean decrease in LV mass and indexed LV mass, and a mean numerical decrease in mean wall thickness in up to 14 out of 18 LV segments. There was also NYHA class reduction in symptoms. Discussion: In this case series, we have shown that substantial weight loss in HCM patients can be associated with a decrease in LV mass, wall thickness and improvement in symptoms. These observations indicate the potential for positive remodeling of the heart by weight loss. Prospective studies of the benefits of weight loss in HCM are needed.

Dilatable Pulmonary Artery Banding Palliation in Congenital Heart Disease.

BACKGROUND: Surgical pulmonary artery banding (PAB) has been limited in practice because of later requirement for surgical removal or adjustment. The aim of this study is to describe our experience creating a dilatable PAB via transcatheter balloon dilation (TCBD) in congenital heart disease (CHD) patients. METHODS: Retrospective chart review of adjustable PAB-outline anatomical variants palliated and patient outcomes. RESULTS: Sixteen patients underwent dilatable PAB-median age 52 days (range 4-215) and weight 3.12 kg (1.65-5.8). Seven (44%) of the patients were premature, 11 (69%) had ventricular septal defect(s) with pulmonary over-circulation, four (25%) atrioventricular septal defects, and four (25%) single ventricle physiology. Subsequent to the index procedure: five patients have undergone intracardiac complete repair, six patients remain well palliated with no additional intervention, and four single ventricles await their next palliation. One patient died from necrotizing enterocolitis (unrelated to PAB) and one patient required a pericardiocentesis postoperatively. Five patients underwent TCBD of the PAB without complication-Two had one TCBD, two had two TCBD, and another had three TCBD. The median change in saturation was 14% (complete range 6-22) and PAB diameter 1.7 mm (complete range 1.1-5.2). Median time from PAB to most recent outpatient follow-up was 868 days (interquartile range 190-1,079). CONCLUSIONS: Our institution has standardized a PAB technique that allows for transcatheter incremental increases in pulmonary blood flow over time. This methodology has proven safe and effective enough to supplant other institutional techniques of limiting pulmonary blood flow in most patients-allowing for interval growth or even serving as the definitive palliation.

ACC/AHA/ASE/HRS/ISACHD/SCAI/SCCT/SCMR/SOPE 2020 Appropriate Use Criteria for Multimodality Imaging During the Follow-Up Care of Patients With Congenital Heart Disease: A Report of the American College of Cardiology Solution Set Oversight Committee and Appropriate Use Criteria Task Force, American Heart Association, American Society of Echocardiography, Heart Rhythm Society, International Society for Adult Congenital Heart Disease, Society for Cardiovascular Angiography and Interventions, Society of Cardiovascular Computed Tomography, Society for Cardiovascular Magnetic Resonance, and Society of Pediatric Echocardiography.

The American College of Cardiology (ACC) collaborated with the American Heart Association, American Society of Echocardiography, Heart Rhythm Society, International Society for Adult Congenital Heart Disease, Society for Cardiovascular Angiography and Interventions, Society of Cardiovascular Computed Tomography, Society for Cardiovascular Magnetic Resonance, and the Society of Pediatric Echocardiography to develop Appropriate Use Criteria (AUC) for multimodality imaging during the follow-up care of patients with congenital heart disease (CHD). This is the first AUC to address cardiac imaging in adult and pediatric patients with established CHD. A number of common patient scenarios (also termed "indications") and associated assumptions and definitions were developed using guidelines, clinical trial data, and expert opinion in the field of CHD.1 The indications relate primarily to evaluation before and after cardiac surgery or catheter-based intervention, and they address routine surveillance as well as evaluation of new-onset signs or symptoms. The writing group developed 324 clinical indications, which they separated into 19 tables according to the type of cardiac lesion. Noninvasive cardiac imaging modalities that could potentially be used for these indications were incorporated into the tables, resulting in a total of 1,035 unique scenarios. These scenarios were presented to a separate, independent panel for rating, with each being scored on a scale of 1 to 9, with 1 to 3 categorized as "Rarely Appropriate," 4 to 6 as "May Be Appropriate," and 7 to 9 as "Appropriate." Forty-four percent of the scenarios were rated as Appropriate, 39% as May Be Appropriate, and 17% as Rarely Appropriate. This AUC document will provide guidance to clinicians in the care of patients with established CHD by identifying the reasonable imaging modality options available for evaluation and surveillance of such patients. It will also serve as an educational and quality improvement tool to identify patterns of care and reduce the number of Rarely Appropriate tests in clinical practice.

Intimal spindle cell sarcoma masquerading as adult-onset symptomatic pulmonic stenosis: a case report and review of the literature.

BACKGROUND: Pulmonary artery intimal spindle cell sarcomas are rare and carry with them a poor prognosis and high rate of recurrence. In extremely rare cases, this tumor can infiltrate the pulmonic valve and manifest as adult-onset pulmonic stenosis. CASE PRESENTATION: We report an unusual case of a patient with symptomatic, adult-onset severe pulmonic stenosis who was referred for possible balloon valvuloplasty but was subsequently found to have pulmonary artery intimal sarcoma infiltrating the pulmonary valve leading to progressive exertional dyspnea. CONCLUSION: The presence of adult-onset pulmonic stenosis should prompt the clinician to investigate further as most cases of pulmonic stenosis are congenital in nature and present early in life. Careful diagnostic evaluation in concert with multimodal imaging should take place to arrive at the correct and challenging diagnosis of sarcoma-induced adult-onset severe pulmonic stenosis. Given the poor prognosis and rapid progression of disease, early diagnosis is crucial.

CT pulmonary angiography of adult pulmonary vascular diseases: Technical considerations and interpretive pitfalls.

Computed tomography pulmonary angiography (CTPA) has become the primary imaging modality for evaluating the pulmonary arteries. Although pulmonary embolism is the primary indication for CTPA, various pulmonary vascular abnormalities can be detected in adults. Knowledge of these disease entities and understanding technical pitfalls that can occur when performing CTPA are essential to enable accurate diagnosis and allow timely management. This review will cover a spectrum of acquired abnormalities including pulmonary embolism due to thrombus and foreign bodies, primary and metastatic tumor involving the pulmonary arteries, pulmonary hypertension, as well as pulmonary artery aneurysms and stenoses. Additionally, methods to overcome technical pitfalls and interventional treatment options will be addressed.

Sequential percutaneous closure of mitral prosthetic paravalvular leak and complex communicating pseudoaneurysms of the ascending aorta and subvalvar left ventricular outflow tract.

Ascending aortic and subvalvar left ventricular outflow tract (LVOT) pseudoaneurysms are rare complications following aortic valve or root replacement surgery. Clinically important paravalvular leaks are rare complications following any valve replacement surgery. We report an unusual case of sequential percutaneous closure of mitral prosthetic paravalvular leak and complex communicating ascending aortic and subvalvar LVOT pseudoaneurysms, which demonstrates the importance of multimodal imaging assessment surrounding percutaneous closure. © 2015 Wiley Periodicals, Inc.

Aorta-to-left atrial fistula developing after surgical removal of an atrial septal occlusion device eight years after original implantation.

Cardiac erosions may occur in a small percentage of patients after device closure of atrial septal defects. These devices have to be explanted. We report an aorta-to-left atrial fistula after surgical explantation of the device eight years after implant for access to mitral valve repair. The importance of realizing the risk of subclinical cardiac erosion and subsequent fistula development after device removal is discussed.

Can we talk? Reflections on effective communication between imager and interventionalist in congenital heart disease.

The rapid proliferation of catheter-mediated treatments for congenital heart defects has brought with it a critical need for cooperation and communication among the numerous physicians supporting these new and complex procedures. New interdependencies between physicians in specialties including cardiac imaging, interventional cardiology, pediatric cardiology, anesthesia, cardiothoracic surgery, and radiology have become apparent, as centers have strived to develop the best systems to foster success. Best practices for congenital heart disease interventions mandate confident and timely input from an individual with excellent adjunctive imaging skills and a thorough understanding of the devices and procedures being used. The imager and interventionalist must share an understanding of what each offers for the procedure, use a common terminology and spatial orientation system, and convey concise and accurate information about what is needed, what is seen, and what cannot be seen. The goal of this article is to review how the cardiovascular imaging specialists and interventionalists can work together effectively to plan and execute catheter interventions for congenital heart disease.

Acute ST changes during anesthesia induction 10 months after Norwood procedure.

After the Norwood procedure for palliation of hypoplastic left heart syndrome, there is still significant interstage and late mortality with often unclear etiology. An important, but possibly under-recognized complication of the Norwood operation is the potential for coronary insufficiency from pre-coronary stenosis due to kinking or scarring at the anastomosis between the native and neo-aorta. We report a case of a clinically thriving 10-month old infant status post bidirectional Glenn who had acute ischemic changes on electrocardiogram (ECG) during induction of anesthesia for elective bilateral herniorrhaphy. A discrete narrowing in the native aorta to neo-aorta anastomosis was identified. A stent was placed emergently to restore adequate coronary blood flow after failure of simple angioplasty to adequately improve the stenosis.

Cyanosis after Kawashima-Fontan: hybrid approach to mixing and matching flows.

Surgical approaches to a single ventricle with absent infrahepatic segment of the inferior vena cava include a bidirectional Glenn and separate hepatic venous connection to the pulmonary arteries. If sites of insertion into the pulmonary arteries are widely separated, hepatic flow is directed to only one lung. Pulmonary arteriovenous malformations may form in the contralateral lung and result in severe cyanosis. We report a case of resolution of pulmonary arteriovenous malformations after successful rerouting of hepatic venous flow to the hemi-azygous vein in a 16-year-old patient. A dilatable pulmonary artery band reduced cyanosis by limiting flow to the affected lung until resolution of pulmonary arteriovenous malformations.

Intermediate to long-term outcome following congenital coronary artery fistulae closure with focus on thrombus formation.

We sought to evaluate intermediate to long-term follow-up after coronary artery fistula (CAF) closure with emphasis on thrombotic complications. Transcatheter closure (TCC) or surgical closure (SC) is the current standard of treatment for significant CAF. Incidence and risk factors of coronary thrombosis after CAF closure have not been well described. Patients with CAF were identified from a departmental database and their medical records were retrospectively reviewed. CAFs were classified as proximal or distal based on origin and size as small, medium, or large. Of 16 patients, 12 underwent TCC and 4 SC. Median follow-up was 2.3 years (0.1 to 41.6). Myocardial infarction (MI) related to coronary thrombosis occurred in 3 patients; immediately, 0.9 year, and 42 years after closure. Ages at MI were 9.2, 57, and 49 years. All 3 had distal, large CAFs and underwent SC. Anticoagulation was used in 2 of 3 patients. In the remaining 13 patients, TCC was performed in 12 and SC in 1; mean age was 13.8 years (0.1 to 38.9). CAFs were proximal in 10 and distal in 3 and large in 10 and medium in 3. On follow-up, these patients were asymptomatic without MI. Anticoagulation was used in 9 of 13 after closure. In conclusion, patients with CAF are at risk for coronary artery thrombosis and MI after closure. Patients with distal type, large CAF, and older age at presentation may be at higher risk for coronary thrombosis. Close follow-up with anatomic and functional coronary evaluation is warranted in all patients after CAF closure.

Transcatheter closure of paraprosthetic valve leaks after surgical mitral and aortic valve replacements.

Prosthetic valve replacement surgery is performed in approximately 210,000 patients annually worldwide. At least 3%, and possibly as many as 12.5%, of these valves will eventually demonstrate a clinically important paravalvular leak. Most of the significantly symptomatic leaks are currently repaired surgically, but the risks of repeat operation and re leak are significantly higher than the original valve-replacement surgical risks. Transcatheter closure of paravalvular leaks has been accomplished using a number of different devices in a relatively small number of centers over the last 16 years. Transcatheter paravalvular leak-closure procedures are still evolving. They are associated with very low procedural mortality rates but are technically challenging, variably effective and may require multiple interventions to be clinically successful. Advances in imaging, such as live 3D transesophageal echocardiography, and the development of better occlusion devices that are specifically designed for closing paravalvular leaks may improve procedure times and outcomes. Further development of effective systems for transcatheter paravalvular leak closure could potentially improve the outlook for a significant population of patients with this difficult and sometimes devastating complication of prosthetic valve replacement.

Maximal potential patent foramen diameter does not correlate with the type or frequency of the neurologic event prior to closure.

BACKGROUND: We analyzed our data on patients undergoing transcatheter patent foramen ovale (PFO) closure to determine if the maximal potential PFO diameter (MPPD) by balloon sizing correlates with important clinical characteristics in this population. METHODS: We defined stroke as a focal neurologic deficit lasting >24 h, or focal deficit of shorter duration associated with permanent MRI/CT changes consistent with a focal infarction. Parameters analyzed included age, gender, anticoagulation, hypertension, smoking, MRI/CT findings and MPPD at catheterization. We specifically analyzed the type of neurologic event (stroke/transient ischemic attack, TIA), and number of recorded preceding clinical neurologic events. RESULTS: In 216 consecutive patients, 167 suffered a stroke. MRI/CT changes consistent with one or more embolic events were seen in 156 patients; 49 had a clinical TIA. There was no significant difference in MPPD between stroke (11.0 +/- 3.6 mm) and TIA groups (10.9 +/- 3.9 mm; 95% confidence interval for difference: -1.33 to 1.00). MPPD did not differ between MRI/CT-positive vs. -negative strokes, and had no correlation with the number of identified pre-closure clinical neurologic events. CONCLUSIONS: Continued investigation is needed to determine whether other PFO characteristics, or other anatomic/physiologic parameters, may be useful to identify patients at high risk for cryptogenic stroke/TIA, even before they have their first neurologic event.

Endovascular stent grafts for large thoracic aneurysms after coarctation repair.

BACKGROUND: Aneurysm formation is a complication not infrequently seen after repair of aortic coarctation and some may enlarge over time. Conventional management of large thoracic aneurysms after aortic coarctation repair has been akin to the surgical treatment of nonspecific aneurysms; however, hypothermic circulatory arrest has been more frequently required because of reoperations. We describe the treatment of a series of patients with large aneurysms using novel endovascular techniques. METHODS: The database of patients undergoing thoracic endograft placement was reviewed to identify those with thoracic aneurysms after aortic coarctation repair. Clinical, operative, and radiographic data were assessed. Follow-up imaging included spiral computed tomography (CT) scans immediately after deployment, at 6 months, and yearly thereafter. RESULTS: Of 9 patients that were identified, 7 presented for elective repair and 2 were emergencies. The aneurysms measured 4.7 to 7.3 cm in diameter on spiral CT scans. Seven patients underwent carotid to subclavian bypass and subclavian ligation. Endografts were placed abutting the origin of the left common carotid artery. Seven patients were treated with Zenith endografts (Cook, Inc, Bloomington, IN), and 2 with TAG devices (W.L. Gore & Associates, Flagstaff, AZ). Left common carotid angioplasty and stenting was performed in 4 patients. No major complications occurred. A mean follow-up of 24 months (range, 6.4 to 48 months) demonstrated no late endoleaks, ruptures, conversions, or migration. CONCLUSIONS: Placement of endovascular stent grafts is a less invasive approach for patients with thoracic aneurysm after aortic coarctation repair, provided there is no residual coarctation or arch hypoplasia. The potential to diminish the magnitude of the surgical procedure and consequences of aortic exposure in a reoperative field is promising and mandates further investigation.

Results of the U.S. multicenter pivotal study of the HELEX septal occluder for percutaneous closure of secundum atrial septal defects.

OBJECTIVES: This study sought to compare the safety and efficacy of the HELEX septal occluder (HSO) with surgical repair of atrial septal defect (ASD). BACKGROUND: The HSO is a low-profile, double-disk occluder device for percutaneous closure of secundum ASD. METHODS: Patients were enrolled (HSO arm prospectively, surgery arm prospectively/retrospectively) from 14 U.S. sites and followed up for 12 months postprocedure. Investigator-reported outcomes were evaluated, including closure success (no or clinically insignificant residual shunt) and the incidence of adverse events. The first 3 HSO patients at each site were considered training cases and were excluded from analysis. RESULTS: Between March 2001 and April 2003, 119 nontraining cases received an HSO and 128 had surgical repair. The groups were similar with statistical but clinically unimportant differences in median age, weight, and preprocedural echocardiographic defect size. Anesthesia time and hospital stay were significantly shorter in the HSO group. Closure success, defined as complete closure or a clinically insignificant residual shunt, was similar in both groups. Major and minor adverse events rates were not statistically different. The most common major adverse events for the HSO group was device embolization requiring catheter retreival (1.7%), and in the surgery group was postpericardiotomy syndrome (6.3%), including one death because of tamponade. The primary end point, clinical success, a composite of closure success and no major adverse events at 12 months, satisfied the noninferiority hypothesis comparing device closure with surgery. CONCLUSIONS: Closure of ASD with the HELEX septal occluder is safe and effective when compared with surgical repair, with reduced anesthesia time and hospital stay. (U.S. Multicenter Pivotal Study of the HELEX Septal Occluder for Percutaneous Closure of Secundum Atrial Septal Defects; this study was approved by the Food and Drug Administration before the National Institutes of Health website was active, so there is not a URL or registration number.).

Selective timing for the arterial switch operation.

BACKGROUND: To determine outcomes for the arterial switch operation individualized according to the underlying anatomy and clinical status. METHODS: A retrospective review of a consecutive series of infants less than 90 days of age who underwent the arterial switch operation at a single institution. RESULTS: From July 1993-April 2001, 117 infants underwent an arterial switch operation before 90 days of age. Seventy-five patients (64%) had transposition of the great arteries with intact ventricular septum with the aim of operation before 14 days of age; however, 8 of these patients had delayed presentation (range 15-46 days). Thirty-five patients (30%) had transposition with a ventricular septal defect (30 patients) or double outlet right ventricle (5 patients) and normal arch anatomy and were repaired within the first 90 days of life depending on the severity of heart failure at a median of 12 days of age (range 3-83 days). Seven patients (6%) had associated aortic coarctation (5 patients) or interrupted aortic arch (2 patients). One patient died during hospitalization (0.85% hospital mortality) and one patient died from noncardiac causes during a median follow-up of 35 months (1.7% total mortality). Four patients required intervention during follow-up (3.4%) for new aortic coarctation (2 patients), supravalvar pulmonic stenosis (1 patient), or right hemi-diaphragm paralysis (1 patient). CONCLUSIONS: Individualized timing for the arterial switch operation within the first ninety days of life produces excellent survival rates for all types of transposition physiology with the expectation of a satisfactory course during follow-up.

Experiência inicial no Brasil com a prótese Helex para oclusão percutânea de defeitos interatriais / Initial experience in Brazil with the Helex septal occluder for percutaneous occlusion of atrial septal defects

OBJECTIVE: To evaluate the initial clinical experience with the Helex septal occluder for percutaneous closure of atrial septal defects. METHODS: Ten patients underwent the procedure, 7 patients with ostium secundum atrial septal defects (ASD) with hemodynamic repercussions and 3 patients with pervious foramen ovale (PFO) and a history of stroke. Mean age was 33.8 years and mean weight was 55.4 kg. Mean diameter by transesophageal echocardiography and mean stretched ASD diameter were 11.33 ± 3.3mm, and 15.2 ± 3.8mm, respectively. The Qp/Qs ratio was 1.9 ± 0.3 in patients with ASD. RESULTS: Eleven occluders were placed because a patient with 2 holes needed 2 devices. It was necessary to retrieve and replace 4 devices in 3 patients. We observed immediate residual shunt (< 2mm) in 4 patients with ASD, and in those with patent foramen ovale total occlusion of the defect occurred. No complications were noted, and all patients were discharged on the following day. After 1 month, 2 patients with ASD experienced trivial residual shunts (1mm). In 1 patient, we observed mild prolapse in the proximal disk in the right atrium, without consequences. CONCLUSION: The Helex septal occluder was safe and effective for occluding small to moderate atrial septal defects. Because the implantation technique is demanding, it requires specific training of the operator. Even so, small technical failures may occur in the beginning of the learning curve, but they do not involve patient safety