Molecular and Genetic Insights into Thoracic Aortic Dilation in Conotruncal Heart Defects

Thoracic aortic dilation (AD) has commonly been described in conotruncal defects (CTDs), such as tetralogy of Fallot, double outlet right ventricle and transposition of the great arteries, and truncus arteriosus. Several theories for this have been devised, but fairly recent data indicate that there is likely an underlying histologic abnormality, similar to that seen in Marfan and other connective tissue disease. The majority of aortic dissection in the general population occurs after the age of 45 years, and there have been very few case reports of aortic dissection in CTD. Given advances in cardiac surgery and increasing survival over the past several decades, there has been rising concern that, as patients who have survived surgical correction of these defects age, there may be increased morbidity and mortality due to aortic dissection and aortic regurgitation. This review discusses the most recent developments in research into AD in CTD, including associated genetic mutations

Evaluation of Left Ventricular Systolic Function after Pulmonary Valve Replacement Using Cardiovascular Magnetic Resonance Imaging

Following reparative surgery for tetralogy of Fallot or critical pulmonary stenosis (PS), patients frequently present with severe right ventricular (RV) volume overload due to pulmonary regurgitation, resulting in decreased RV function. Surgical pulmonary valve replacement (PVR) is known to improve RV function, but changes in left ventricular (LV) function after PVR have rarely been described. We sought to determine the midterm results regarding LV systolic function after PVR using cardiac MRI in 40 consecutive patients with repaired TOF (31 patients) or PS (9 patients) with an age of 29±9 years who underwent PVR from 2006 to 2011 at a single center. Cardiac MRI RV and LV volumes before and after PVR were analyzed. Demographics, clinical variables, cardiopulmonary bypass duration, and medications were reviewed. LV ejection fraction (LVEF) increased from (54±8) to (57±6)% (P=0.02). Before PVR, 26 patients had depressed LVEF of (49±5)% (range 36–54%). In this group, LVEF increased by (7±7)% (P<0.0001) after PVR. Low LVEF before PVR was correlated with increased LVEF after PVR (regression coefficient −0.7, R 2 =0.59, P<0.0001). Demographics, medications, prior pregnancies, and cardiopulmonary bypass duration had no effect on LVEF after PVR. The increase in LVEF was most significant in patients with low pre-PVR LVEF

Converting Fontan-Björk to 1.5- or 2-ventricle circulation

Patients with tricuspid atresia and ventricular septal defect have in the past occasionally undergone a Fontan with “Björk” modification to create a connection between the right atrium and the right ventricular outflow tract. While rarely performed now, patients with this physiology often face severe complications requiring re-intervention. We hypothesize that surgical conversion to a 2-ventricle or 1.5-ventricle circulation can improve hemodynamics, clinical status, and thus increase time to transplant. We present two successful cases to illustrate this idea

Permanent Nonselective His Bundle Pacing in an Adult with L-Transposition of the Great Arteries and Complete AV Block

We report the placement of a permanent transvenous nonselective His bundle pacing lead in conjunction with a transvenous pacemaker/implantable cardioverter-defibrillator in an adult with Levo-Transposition of the Great Arteries (L-TGA) and a stenotic coronary sinus (CS) ostium, which would not accommodate a transvenous left ventricular (LV) pacing lead. Nonselective His bundle pacing provided a nearly identical ventricular activation pattern in this previously unpaced patient. Many L-TGA patients will have an eventual need for permanent pacing and, given the challenges of CS cannulation, His bundle pacing may represent a preferred modality rather than pure morphologic LV pacing or surgical systemic ventricular lead placement to achieve optimal electrical synchrony

Non-invasive cardiac output monitoring (NICOM) in adult congenital heart disease patients with Fontan palliation

Rationale Fontan palliation for single ventricle malformations is an increasingly common reason for heart failure in the adult population. Cardiac output (CO) measurement in Fontan physiology is achieved by invasive cardiac catheterization (RHC). Noninvasive CO monitors using thoracic bioreactance (NICOM) have been validated in non-congenital patients but have not been studied in adult Fontan patients. Objective To compare RHC obtained values of CO using the Fick equation with those measured simultaneously by NICOM in a cohort of adults with Fontan palliation. Methods and results In nineteen patients undergoing routine outpatient RHC, we compared CO values as determined by Fick with those generated by the Starling SV NICOM device. Bland-Altman plots and intraclass correlation coefficients (ICCs) revealed internal consistency within NICOM measurements, however the agreement between RHC and NICOM for CO was poor (ICCs ​∼ ​0.40). We performed sub-analyses using two-sample T-tests and ICCs to determine if clinical cyanosis, acute desaturation, or Fontan pressure affected the difference observed between RHC and NICOM. Neither chronic hypoxia, acute desaturation, nor Fontan pressure measures were found to be associated with the observed difference between the RHC and NICOM measured CO. Discussion and conclusion Our study did not find a correlation between RHC and NICOM derived measures of CO in a cohort of Fontan patients, even in sub-analyses of confounders of Fontan physiology. We observed internal consistency within the device, which may open a role for monitoring of trends rather than absolute values in Fontan patients. Our study was limited due to small sample size

Long Term Consequences of the Fontan Procedure and How to Manage Them

In 1971, Fontan and Baudet described a surgical technique for successful palliation of patients with tricuspid atresia. Subsequently, this technique has been applied to treat most forms of functional single ventricles and has become the current standard of care for long-term palliation of all patients with single ventricle congenital heart disease. Since 1971, the Fontan procedure has undergone several variations. These patients require lifelong management including a thorough knowledge of their anatomic substrate, hemodynamic status, management of rhythm and ventricular function along with multi organ evaluation. As these patients enter middle age, there is increasing awareness regarding the long-term complications and mortality. This review highlights the long-term outcomes of the Fontan procedure and management of late sequelae

Successful TPV Implantation in a Pregnant Patient With Right Ventricle to Pulmonary Artery Conduit Obstruction

A patient with repaired double outlet right ventricle presented during early gestation with heart failure symptoms due to severe right ventricle–pulmonary artery conduit stenosis and insufficiency. In the first trimester, she underwent transcatheter therapy with Melody pulmonary valve implantation with excellent hemodynamic results and completed pregnancy without significant maternal complications. (Level of Difficulty: Advanced.

Management of Severe Coarctation of the Aorta During Pregnancy

An 18-year-old primigravida woman underwent emergent percutaneous balloon stent repair of a severe coarctation of the aorta, allowing her to undergo an uneventful remaining pregnancy and the delivery of a healthy baby. Her case also demonstrates the association between maternal coarctation and fetal Shone complex. (Level of Difficulty: Beginner.

Heart Transplantation in Mustard Patients Bridged With Continuous Flow Systemic Ventricular Assist Device - A Case Report and Review of Literature

Thirty four-year-old male with history of D-transposition of the great arteries (D-TGA) who underwent Mustard operation at 14 months of age presented in cardiogenic shock secondary to severe systemic right ventricular failure. Catheterization revealed significantly increased pulmonary pressures. Due to the patient's inotrope dependence and prohibitive pulmonary hypertension, he underwent implantation of a Heart Ware HVAD® for systemic RV support. Within 4 months of continuous flow ventricular assist device (VAD) implantation complete normalization of pulmonary vascular resistance (PVR) was achieved. He ultimately underwent orthotopic heart transplantation with favorable outcomes. This is the second report of complete normalization of PVR following VAD implantation into a systemic RV in <4 months. We conducted a thorough literature review to identify Mustard patients that received systemic RV VAD as a bridge to a successful heart transplantation. In this article, we summarize the outcomes and focus on pulmonary hypertension reversibility following VAD implant

Julio C. TREBOLLÉ-BARRERA, Jehú y Joás. Texto y composición literaria de 2 Reyes 9-11, Edilva («Institución San Jerónimo», 17), Valencia 1984, 254 pp., 16 x 24. [RECENSIÓN]

Peer Reviewedhttps://deepblue.lib.umich.edu/bitstream/2027.42/147202/1/jgc40689.pd