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We report an unusual case of an aorta to left ventricular communication in a neonate presenting with cardiac failure. Although extremely rare, this defect is the most common cause of abnormal blood flow from the aorta to a ventricle in infancy. Early diagnosis and prompt surgical repair are critical to management and results are generally excellent. Long-term follow-up is required to monitor for aortic insufficiency.

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Ventricular tachycardia and cardiac tumors are both extremely rare diagnoses in pediatric patients. We report a pediatric case of cardiac fibroma that was noted during the work up of ventricular tachycardia in a young patient concomitantly diagnosed with severe acute respiratory syndrome coronavirus 2.

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Arrhythmogenic right ventricular cardiomyopathy (ARVC) is a rare, genetically-inherited cardiomyopathy that may be fatal. We present the case of a 17 year old male who presented after a witnessed cardiac arrest with indeterminate echocardiogram and electrocardiogram (ECG) findings for a specific etiology. Genetic testing revealed a mutation in the PKP2 and DSC2 genes, consistent with ARVC. This report outlines the presentation of ARVC as an aborted sudden cardiac death episode in a previously asymptomatic teenager, investigations for ARVC and highlights the importance of adequate cardiopulmonary resuscitation in the overall prognosis. Implantable cardiac defibrillator (ICD) placement for secondary prevention is necessary.

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Mitral valve prolapse (MVP) is a common and predominantly benign condition; however, occasional reports of cardiac arrest in individuals with MVP have raised concern for a malignant subtype. The risk of sudden cardiac arrest in MVP is twice as high as that in the general population. The exact aetiology and risk predictors are elusive, but identification is necessary, if we are to protect patients at risk for cardiac arrest. This report highlights cardiac arrest as the initial presenting symptom of MVP. Rapid initiation of bystander cardiopulmonary resuscitation and time to first shock are key predictors of prognosis. Better screening to identify individuals with malignant phenotypes may aid in reducing the morbidity and mortality in patients with a predisposition for life-threatening arrhythmias.

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BACKGROUND: Aortic root translocation (Nikaidoh), Rastelli, and réparation à l'etage ventriculaire (REV) are repair options for transposition of the great arteries (TGA) with ventricular septal defects and left ventricular outflow tract obstruction (VSD-LVOTO) or double outlet right ventricle TGA type (DORV-TGA). METHODS: This retrospective study using The Society of Thoracic Surgeons Congenital Heart Surgery Database evaluates surgical procedure utilization and outcomes of patients undergoing repair of TGA-VSD-LVOTO and DORV-TGA with a Nikaidoh, Rastelli, or REV procedure. RESULTS: A total of 293 patients underwent repair at 82 centers (January 2010 to June 2019). Most patients underwent a Rastelli (n = 165, 56.3%) or a Nikaidoh (n = 119, 40.6%) operation; only 3.1% (n = 9) underwent a REV. High-volume centers performed the majority of the repairs. Fewer Nikaidoh than Rastelli patients had prior cardiac operations (n = 57 [48.7%] vs n = 102 [63.0%]; P = .004). Nikaidohs had longer median cardiopulmonary bypass time (227 [interquartile range (IQR), 167-299] minutes vs 175 [IQR, 133-225] minutes; P < .001) and median aortic cross-clamp times (131 [IQR, 91-175] minutes vs 105 [IQR, 82-141] minutes; P = .0015). Operative mortality was 3.1% (95% confidence interval [CI], 1.0%-7.0%; n = 5) for Rastelli, 4.4% (95% CI, 1.4%-9.9%; n = 5) for Nikaidoh, and 11.1% (95% CI, 0.3%-48.3%, n = 1) for REV. The rates of cardiac arrest, unplanned reoperation, mechanical circulatory support, prolonged ventilation, and permanent pacemaker placement were higher in the Nikaidoh population but with 95% CIs overlapping those of the other procedures. CONCLUSIONS: Rastelli and Nikaidoh procedures are the prevalent repair strategies for patients with DORV-TGA and TGA-VSD-LVOTO. Most are performed at high-volume institutions, and early outcomes are similar.

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Extracorporeal life support (ECLS) is an essential component of a modern congenital cardiac surgery program. The circuit components and bedside management team may, however, vary among institutions. Here, we evaluate our initial experience with a modified ventricular assist device-based ECLS circuit primarily managed by the bedside nurse. We hypothesize that our outcomes are comparable to Extracorporeal Life Support Organization (ELSO) registry data. All patients who received ECLS from January 1, 2016 to December 31, 2019 at a single institution were included. Primary outcomes were survival to ECLS decannulation and discharge or transfer. Secondary outcomes included complications from ECLS. Data were compared to available ELSO registry data. Thirty-seven patients underwent 44 ECLS runs during the study period. Forty percent of patients had single ventricle physiology. Nearly 46% of patients received ECLS as part of extracorporeal cardiopulmonary resuscitation (eCPR). Survival to ECLS decannulation (68.2%) and survival to discharge or transfer (61.4%) did not differ from overall ELSO outcomes (69.7%, P = .870 and 50.7%, P = .136), as well as survival to discharge or transfer in a comparable cohort of ELSO centers (53.1%, P = .081). Patients with complications had a lower rate of survival to discharge or transfer but this did not reach statistical significance (47.7% vs. 75.0%, P = .455). Neurologic (50.0%), hemorrhagic (45.5%), and renal complications (31.8%) were most common in this cohort. A modified ventricular assist device-based ECLS circuit with primary management by the bedside nurse can provide comparable support in a neonatal and pediatric cardiac surgery population. Cost analyses and further delineation of the complication profile are necessary for a complete characterization of this system.

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TandemHeart, an adult ventricular assist device, is also being used in children for mechanical circulatory support. In this case series, we describe our experience using TandemHeart ventricular assist device with a modified circuit to provide mechanical circulatory support in three neonates for multiple indications. TandemHeart ventricular assist device with a modified circuit can be used successfully to provide extracorporeal support to neonates with complex CHD.

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A cardiac papillary fibroelastoma is a benign, pedunculated tumor with strand-like projections arising from the endocardium. While often discovered incidentally, these tumors can embolize resulting in major neurologic events. There is a dearth of pediatric literature describing the necessity and optimal timing of surgical intervention for this rare primary cardiac tumor after a cerebrovascular event. We report an interesting case and the subsequent management dilemma in a teenage patient presenting with acute embolic strokes secondary to an aortic valve papillary fibroelastoma. Despite its rarity, a high index of suspicion should be maintained for such tumors in previously healthy children presenting with acute stroke.

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We describe a case of spontaneous hyphema presentation in an infant who underwent repair of tetralogy of Fallot. This case illustrates a previously unreported cause of hyphema formation from a combination of venous congestion caused by elevated right ventricular pressure and residual coagulopathy after cardiopulmonary bypass.

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Hypoplastic left heart syndrome is a congenital heart defect characterized by hypoplasia of left heart structures. Over the past 3 decades, there have been advances in techniques and management in the care of these patients. We discuss shunt selection, operative strategies, transplantation, and outline various facets of management at the University of Florida. This is a review of the literature highlighting some of the technical aspects of the preoperative, operative, and postoperative care that are important in today's management of hypoplastic left heart syndrome.

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BACKGROUND: Surgical reconstruction is the primary method of treating airway obstruction in children. Tracheal stenosis is frequently associated with congenital heart defects, which may further complicate the overall management strategy. The purpose of this study was to review our experience with surgical reconstruction of airway obstruction in conjunction with congenital heart defects. METHODS: This was a retrospective review of our surgical experience with tracheal stenosis from February 2003 to August 2011. Twenty-seven patients were identified in our database. Six patients had isolated, congenital tracheal stenosis, and 21 had tracheal stenosis in association with congenital heart defects. There were two identifiable subgroups. Thirteen patients had airway stenoses identified concurrently with congenital heart defects and underwent combined repair. The second group comprised 8 patients who had previous correction of their congenital heart defects and experienced delayed presentation of tracheal (n = 6) or bronchial (n = 2) obstruction. RESULTS: The median age at surgery was 9 months. There were 2 postoperative deaths, both in children with single ventricle. The median duration of follow-up for the entire cohort of 25 surviving patients was 4 years. None of the patients have required reoperations on the trachea; 5 have had minor reinterventions. CONCLUSIONS: The data demonstrate that tracheal obstruction is frequently found in conjunction with congenital heart defects. Nearly one third of our patients had delayed presentation of airway obstruction that was identified subsequent to previous congenital heart defect repair. Tracheal reconstructive techniques were effective regardless of the cause of the airway obstruction.

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Pacemaker and implantable cardioverter defibrillator (ICD) leads are placed routinely with few notable complications. A rarely described complication of transvenous lead placement is malpositioning into the left ventricle. This situation can cause additional complications in the form of thromboembolic events. We present a case of a malpositioned left ventricular lead that was successfully removed with a minimally invasive technique.

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Duchenne muscular dystrophy (DMD) is a fatal disease of striated muscle deterioration caused by lack of the cytoskeletal protein dystrophin. Dystrophin deficiency causes muscle membrane instability, skeletal muscle wasting, cardiomyopathy, and heart failure. Advances in palliative respiratory care have increased the incidence of heart disease in DMD patients, for which there is no cure or effective therapy. Here we have shown that chronic infusion of membrane-sealing poloxamer to severely affected dystrophic dogs reduced myocardial fibrosis, blocked increased serum cardiac troponin I (cTnI) and brain type natriuretic peptide (BNP), and fully prevented left-ventricular remodeling. Mechanistically, we observed a markedly greater primary defect of reduced cell compliance in dystrophic canine myocytes than in the mildly affected mdx mouse myocytes, and this was associated with a lack of utrophin upregulation in the dystrophic canine cardiac myocytes. Interestingly, after chronic poloxamer treatment, the poor compliance of isolated canine myocytes remained evident, but this could be restored to normal upon direct application of poloxamer. Collectively, these findings indicate that dystrophin and utrophin are critical to membrane stability-dependent cardiac myocyte mechanical compliance and that poloxamer confers a highly effective membrane-stabilizing chemical surrogate in dystrophin/utrophin deficiency. We propose that membrane sealant therapy is a potential treatment modality for DMD heart disease and possibly other disorders with membrane defect etiologies.

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Schwannomas are relatively uncommon, benign nerve sheath tumors. Thoracic schwannomas most often appear in the posterior mediastinum. Pulmonary schwannomas are exceedingly rare and can present a diagnostic challenge. We present a case of an endobronchial schwannoma presenting with bronchial obstruction and review the literature of this unusual entity.

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Relaxation abnormalities are prevalent in heart failure and contribute to clinical outcomes. Disruption of Ca2+ homeostasis in heart failure delays relaxation by prolonging the intracellular Ca2+ transient. We sought to speed cardiac relaxation in vivo by cardiac-directed transgene expression of parvalbumin (Parv), a cytosolic Ca2+ buffer normally expressed in fast-twitch skeletal muscle. A key feature of Parv's function resides in its Ca2+/Mg2+ binding affinities that account for delayed Ca2+ buffering in response to the intracellular Ca2+ transient. Cardiac Parv expression decreased sarcoplasmic reticulum Ca2+ content without otherwise altering intracellular Ca2+ homeostasis. At high physiological mouse heart rates in vivo, Parv modestly accelerated relaxation without affecting cardiac morphology or systolic function. Ex vivo pacing of the isolated heart revealed a marked heart rate dependence of Parv's delayed Ca2+ buffering effects on myocardial performance. As the pacing frequency was lowered (7 to 2.5 Hz), the relaxation rates increased in Parv hearts. However, as pacing rates approached the dynamic range in humans, Parv hearts demonstrated decreased contractility, consistent with Parv buffering systolic Ca2+. Mathematical modeling and in vitro studies provide the underlying mechanism responsible for the frequency-dependent fractional Ca2+ buffering action of Parv. Future studies directed toward refining the dose and frequency-response relationships of Parv in the heart or engineering novel Parv-based Ca2+ buffers with modified Mg2+ and Ca2+ affinities to limit systolic Ca2+ buffering may hold promise for the development of new therapies to remediate relaxation abnormalities in heart failure.

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BACKGROUND: Despite acceptable results reported with endovascular thoracic aortic repair (TEVAR), recent studies have questioned the merit of repair in asymptomatic patients considered high risk for open surgery. In this group, advanced age or comorbid conditions may reduce life expectancy, thus limiting the benefit of elective aneurysmectomy. This study was conducted to determine whether elective TEVAR improves survival for this cohort. METHODS: Forty-six asymptomatic patients with descending thoracic aortic disease were considered high risk for open surgery for reasons of age of 80 years or older (47.8%) or comorbid conditions (84.8%), and were subsequently evaluated for elective TEVAR. Of these, 21 underwent TEVAR, while another 25 patients were excluded from TEVAR on the basis of unfavorable anatomy or refused intervention. RESULTS: The mean age of the cohort was 77.0 +/- 7.0 years (p = 0.9 between groups). Prevalent comorbid conditions were similar between groups, and included coronary artery disease (p = 1.0), chronic obstructive pulmonary disease (p = 1.0), and peripheral vascular disease (p = 0.23). Mean maximum aortic diameter was 6.0 +/- 1.4 cm (p = 0.54 between groups). Indications for intervention included fusiform aneurysm (65.2%) and pseudoaneurysm or penetrating ulcer (32.6%). No 30-day mortality was observed after TEVAR. All-cause mortality in the entire cohort was 50%. Median actual time to mortality was different between groups (control, 9.2 months versus TEVAR, 24.9 months; p = 0.01). Life-table analysis demonstrated improved survival for TEVAR at 24 months (p = 0.05). CONCLUSIONS: Although the overall prognosis for the asymptomatic patient with descending thoracic aortic disease at high risk for open surgery is poor, elective endovascular repair improves survival and should be considered a therapeutic option in this setting.

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BACKGROUND: Endovascular approaches promise to revolutionize therapy for thoracic aortic disease. This study describes a long-term analysis of endovascular thoracic aortic repair. METHODS: Seventy-three patients (mean age, 67.4 years) underwent endovascular thoracic aortic repair from 1993 to 2005. Indications for intervention included aneurysm (38%), dissection (23%), or penetrating ulcer or pseudoaneurysm (34%). Rupture was present in 16 patients (22%). Seventy-one percent were considered high risk for open surgery for reasons of age or comorbid conditions. Treated segments included ascending aorta (n = 1), distal arch (n = 24), and proximal (n = 50) or distal (n = 55) descending aorta. The total descending thoracic aorta was covered in 31 patients. Procedural success was achieved in 96%. Devices were delivered by femoral (79%), retroperitoneal iliac (18%), or carotid (2.7%) exposure. Devices used included Excluder (n = 30), Talent (n = 23), Zenith (n = 3), AneuRx (n = 5), and custom-fabricated (n = 14). Follow-up was 100% complete. RESULTS: Thirty-day mortality was 5.5%. Significant morbidity included stroke (8.2%) and need for dialysis (4.1%). Although 3 patients had transient spinal cord ischemia (4.1%), none had permanent sequelae. Intervention for fusiform aneurysm was independently associated with a composite end point of 30-day mortality, need for dialysis, and stroke (p = 0.015). Eight patients (11%) had new or persistent endoleaks, and aortic reintervention was performed in 7 patients (9.6%). Mean survival for the entire cohort was 46.8 +/- 5.1 months. Intervention for penetrating ulcer or pseudoaneurysm (p = 0.045) was independently associated with long-term all-cause mortality. CONCLUSIONS: An endovascular approach produces acceptable results for a broad range of thoracic aortic disease. However, the potential for endoleak or need for reintervention mandates continued close follow-up to achieve satisfactory long-term results.

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BACKGROUND: Use of hypothermic circulatory arrest (HCA) for operations on the descending thoracic aorta is controversial. While deep hypothermia may provide better end-organ and spinal cord function, prolonged cardiopulmonary bypass and circulatory arrest may increase morbidity. This study assessed outcomes after use of HCA for descending thoracic aortic resection in a large cohort of consecutive patients. METHODS: Hypothermic circulatory arrest was utilized if arch or extensive descending thoracic aortic resection was required, or if aortic pathology precluded cross-clamping. One hundred thirty-two patients (mean age, 61.3 years) were identified. Diagnosis included fusiform (41.2%) or saccular aneurysm (10.7%) and acute (4.6%) or chronic (38.9%) dissection. Twenty-one patients presented with rupture. Arch resection (distal arch 100, total arch 11) was required in 111 patients (84.1%). The extent of descending thoracic aortic resection (required in 94%) included proximal third in 41 patients, proximal two-thirds in 6, and complete thoracic aorta in 77. The proximal anastomosis was performed with total body HCA while the distal anastomosis was constructed with lower body HCA only (duration upper body HCA 33.7 +/- 8.0 minutes; total duration lower body HCA 71.3 +/- 24.2 minutes). RESULTS: Thirty-day mortality was 6.0%. Neurologic events included stroke (6.8%) and permanent lower extremity paralysis-paresis (4.5%). Temporary dialysis was needed in 7 (5.3%), though only 2 patients required permanent dialysis (1.9%). Independent predictors of a composite endpoint of death, stroke, permanent paralysis, or dialysis included duration of lower body HCA (p = 0.03) and major postoperative infection (p = 0.003). CONCLUSIONS: Adjunctive use of deep hypothermic circulatory arrest for descending thoracic aortic resection affords excellent preservation of end-organ and spinal cord function with acceptable rates of mortality and significant morbidity.

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