Browsing by Author "Kay, W. Aaron"
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Item Converting Fontan-Björk to 1.5- or 2-ventricle circulation(Elsevier, 2018) Hopkins, Kali A.; Brown, John W.; Darragh, Robert K.; Kay, W. Aaron; Graduate Medical EducationPatients 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.Item Long Term Consequences of the Fontan Procedure and How to Manage Them(Elsevier, 2018) Kay, W. Aaron; Moe, Tabitha; Suter, Blair; Tennancour, Andrea; Chan, Alice; Krasuski, Richard A.; Zaidi, Ali N.; Medicine, School of MedicineIn 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.Item Management of Coarctation of the Aorta during Pregnancy: A Case Report.(2019-07) Patel, Pooja; Ciresi, Colette; Asdell, Stephanie M.; Hopkins, Kali; Kay, W. AaronBACKGROUND: Patients with coarctation of the aorta are increasingly reaching child-bearing age. Unrepaired coarctations and/or repaired coarctations with recurrent or residual stenosis require close monitoring for hypertension and heart failure, which can increase maternal risk for aortic rupture or dissection and neonatal risks for intrauterine growth retardation, abruptio placentae, and premature delivery. The potential for adverse outcomes may necessitate repair during pregnancy. CASE: An 18-year-old primigravida at 11 weeks gestation with history significant for unrepaired coarctation of the aorta presented with chest pain for 2-3 days and nonpalpable lower extremity pulses. She was previously diagnosed with coarctation at age 3 years, which did not require surgical repair and she was subsequently lost to follow up. Echocardiogram (ECHO) showed a dilated ascending and transverse aorta with decreased pulsatility in the descending aorta, consistent with critical coarctation. Emergent cardiac catheterization was performed and a balloon expanded stent was used to dilate the area of coarctation. Post-procedurally she had zero gradient and went on to have an uneventful pregnancy and delivery. She is now a 23-year-old G5P3. She has two living children with Shone’s complex and had two pregnancies end in miscarriages. Fetal ECHO was performed for each pregnancy and the patient received genetic counseling but declined further genetic testing. CLINICAL SIGNIFICANCE: This case illustrates a successful percutaneous repair of coarctation during pregnancy via balloon expanded stent placement. Fetal ECHO should occur from 18-22 weeks gestation in pregnant women with history of coarctation as prevalence of left-sided obstructive lesions may occur in 10–20% of first-degree relatives. As shared cardiac genetic markers exist between coarctation and Shone’s complex, this case also demonstrates the impact prenatal genetic testing can potentially have on subsequent pregnancies and neonatal outcomes.Item Management of Severe Coarctation of the Aorta During Pregnancy(Elsevier, 2020-01) Ciresi, Colette M.; Patel, Pooja R.; Asdell, Stephanie M.; Hopkins, Kali A.; Hoyer, Mark H.; Kay, W. Aaron; Pediatrics, School of MedicineAn 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.)Item Molecular and Genetic Insights into Thoracic Aortic Dilation in Conotruncal Heart Defects(Frontiers Media SA, 2016) Kay, W. Aaron; Department of Medicine, IU School of MedicineThoracic 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.Item Non-invasive cardiac output monitoring (NICOM) in adult congenital heart disease patients with Fontan palliation(Elsevier, 2021-12) Quain, Angela; Hoyer, Mark; Ephrem, Georges; Kay, W. Aaron; Medicine, School of MedicineRationale 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.Item Permanent Nonselective His Bundle Pacing in an Adult with L-Transposition of the Great Arteries and Complete AV Block(Wiley, 2017) Kean, Adam C.; Kay, W. Aaron; Patel, Jyoti K.; Miller, John M.; Dandamudi, Gopi; Department of Pediatrics, IU School of MedicineWe 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.Item Successful TPV Implantation in a Pregnant Patient With Right Ventricle to Pulmonary Artery Conduit Obstruction(Elsevier, 2020-01) Detzner, Ashley A.; Hopkins, Kali A.; Kay, W. Aaron; Hoyer, Mark H.; Pediatrics, School of MedicineA 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.)Item Warden Procedure in a 77-year-old Man(Elsevier, 2019) Hopkins, Kali A.; Farber, Mark O.; Singh, Rajeev; Kay, W. Aaron; Herrmann, Jeremy L.; Brown, John W.; Medicine, School of MedicinePartial anomalous pulmonary venous return (PAPVR) is a rare congenital heart defect characterized by one or more but not all of the pulmonary veins draining somewhere other than the left atrium thereby creating a left-to-right shunt. Over time, patients may develop right-sided volume overload and its subsequent complications. We present a case of isolated PAPVR in an older patient who underwent a Warden procedure at age 77 years with rapid improvement in right ventricular size and function.