SESSION TYPE: Cardiovascular CasesPRESENTED ON: Wednesday, October 24, 2012 at 11:15 AM - 12:30 PM

INTRODUCTION:

Severe hypoxemia is commonly encountered by physicians. In the absence of significant parenchymal lung disease, the differential is limited and includes disorders such as pulmonary embolism, pulmonary vascular disease, and either intrapulmonary or intracardiac shunts. We describe a case of a patient with a history of porcine mitral valve replacement, who presented with severe hypoxemia with clear lung fields on chest imaging. She was found to have a large right to left shunt secondary to a right pulmonary artery to superior pulmonary vein fistula.

CASE PRESENTATION:

A 71-year-old female with a history of coronary artery bypass grafting and porcine mitral valve replacement in 2001 for severe mitral stenosis, presented with dyspnea. Her examination was remarkable for severe hypoxia and bilateral lower extremity edema. Pertinent laboratory data was remarkable for the following: hemoglobin 14.7 g/dL, brain natriuretic peptide of 2,590 pg/ml, and a PaO2 of 38 mm Hg on 100% nonrebreather mask. A chest roentogram showed clear lung fields bilaterally. Evaluation with a contrast enhanced chest CT was negative for acute pulmonary embolism or parenchymal pathology. A transthoracic echocardiogram showed right ventricular and left atrial dilatation, a functional bioprosthetic mitral valve, and a positive bubble study after the third heart beat. A transesophageal echocardiogram confirmed the positive bubble study, and did not show an atrial or ventricular septal defect. Right heart catheterization showed a mean pulmonary arterial pressure of 20 mm Hg, a pulmonary capillary wedge pressure of 12 mm Hg, and a pulmonary vascular resistance of 1.3 woods units. No left to right shunt was detected. Re-evaluation of the previous chest CT by the interventional radiologist revealed the presence of a large right pulmonary artery to superior pulmonary vein fistula, which was thought to be related to her previous mitral valve surgery. She underwent placement of an Amplatzer duct occluder with subsequent improvement in both symptoms and oxygenation.

DISCUSSION:

Pulmonary arteriovenous malformations (PAVMs) are vascular communications between pulmonary arteries and veins. While the majority of PAVMs are congenital in nature, they have also been associated with other medical conditions, such as liver cirrhosis, mitral stenosis, and congenital heart disease. To the best of our knowledge, this is the only case that describes the presence of a large pulmonary arteriovenous fistula that may be related to mitral valve replacement and coronary artery bypass grafting.

CONCLUSIONS:

This case illustrates the importance of evaluating patients for underlying pulmonary vascular shunts in the setting of unexplained hypoxemia.1) Gossage JR, Kanj G. Pulmonary Arteriovenous Malformations: A State of the Art Review. Am J Respir Crit Care Med 1998; 158: 643-661DISCLOSURE: The following authors have nothing to disclose: Anthony Cucci, Rajat Gupta, Sandeep Kukreja, Khalil DiabNo Product/Research Disclosure InformationIndiana University, Indianapolis, IN.

SESSION TYPE: Cardiovascular Student/Resident Case Report Posters IIPRESENTED ON: Tuesday, October 23, 2012 at 01:30 PM - 02:30 PM

INTRODUCTION:

Atrial septal defect (ASD) is the most common type of congenital heart disease in children, discovered incidentally in adults with complications like pulmonary hypertension. Their manifestation during pregnancy carries high risk of mortality and hence pregnancy is contraindicated.

CASE PRESENTATION:

31 year old pregnant woman (38 weeks) with no significant past medical history and three previous normal pregnancies presents in active labor and had a normal vaginal delivery. During sterilization procedure she was hypoxic with oxygen saturation around 88% that improved with supplemental oxygen. She was totally asymptomatic. Physical examination then revealed normal S1, a fixed split of S2 and soft systolic ejection murmur. Lungs were clear to auscultation. Chest xray and CT scan of chest showed enlarged main pulmonary arteries, central and interlobar arteries. Connective tissue disease work up was negative and HIV test non-reactive. She had no family history of heart problems or sudden cardiac death. She denied smoking and drugs. Echocardiography with bubble study uncovered the interatrial shunt and RV systolic pressures of 80-90mm of Hg. Transesophageal echocardiography showed large atrial septal defect (ASD) measuring 2.9x2 cm with bidirectional flow, predominantly left to right with calculated qp:qs 2.3:1. 6 weeks post pregnancy she had a right heart catheterization which showed mean pulmonary artery pressure of 61mm of Hg and borderline significant vasoreactivity to adenosine challenge. She was diagnosed of Type I Pulmonary Arterial Hypertension secondary to Ostium Secundum type of ASD. Prophylactic measures included wearing knee length stockings continuously and filtering all her IVs during hospitalization. Screening of her first degree relatives was initiated. She was referred for ASD closure.

DISCUSSION:

Pulmonary Hypertension is defined as elevated mean pulmonary artery pressure greater than 25mm of Hg at rest. Type I pulmonary artery hypertension (PAH) is thought to be secondary to disease affecting the small pulmonary muscular arterioles and includes congenital heart defects, connective tissue disease, HIV infection and drug effects. Previously undiagnosed congenital heart defects like ASD could lead to PAH with decreased functional status and enhanced mortality due to Eisenmengarization. Hemodynamic changes during pregnancy like increased blood volume and pressure changes can lead to shunt reversal which is more catastrophic. When pregnant women with congenital heart disease have an initial successful pregnancy, the long-term ill effects are generally not cumulative.

CONCLUSIONS:

Successive pregnancies generally entail the same but not greater risk. Likewise our patient had not one, nor two, but four normal pregnancies.1) Child, JS. Management of Pregnancy and contraception in Congenital Heart Disease. In: Congenital Heart Disease in Adults, 3rd ed, Perloff, JK, Child, JS, Aboulhosn, J (Eds), Saunders/Elsevier, 2009DISCLOSURE: The following authors have nothing to disclose: Sashank Kolli, Bhavith Aruni, Clifton ClarkeNo Product/Research Disclosure InformationAdvocate Illinois Masonic Medical Center, Chicago, IL.

SESSION TYPE: DVT/PE/Pulmonary Hypertension Posters IIPRESENTED ON: Wednesday, October 24, 2012 at 01:30 PM - 02:30 PM

PURPOSE:

Pulmonary arterial hypertension (PAH) is a rapidly progressive disease with significant morbidity and mortality, particularly when refractory to medical therapy or during acute decompensation; these patients may require lung transplantation but often die waiting. We describe a single center experience of 3 patients with severe group 1 PAH, refractory to targeted medical therapy, in which awake ECMO was successfully used as a bridge to recovery or lung transplantation.

METHODS:

Patient #1, a 22 year-old woman with severe PAH and an atrial septal defect (ASD), had progressive right heart failure and hypoxemic respiratory failure associated with hemoptysis, despite maximal PAH therapy. Venovenous ECMO was initiated with catheter-directed blood flow across the ASD via the internal jugular vein as bridge to transplantation. Patient #2, a 22 year-old woman with surgically corrected transposition of the great vessels and PAH, developed an acute respiratory infection leading to refractory hypoxemic respiratory failure. She was placed on upper-body venoarterial ECMO as bridge to recovery. Patient #3, a 34 year-old woman with PAH and decompensated right heart failure despite maximal PAH therapy, was placed on upper-body venoarterial ECMO as bridge to transplantation.

RESULTS:

All 3 patients were extubated within 24 hours of ECMO initiation. The upper-body ECMO configuration allowed for daily physical therapy, including ambulation of up to 2400 feet. Patient #1 underwent lung transplantation on ECMO day #7 and is now 18 months post-transplant and fully independent. Patient #2 recovered from her acute illness, was decannulated after 8 days using IV epoprostenol to bridge off ECMO, and remains only on oral therapy. Patient #3 underwent lung transplantation on ECMO day #19 and is expected to fully recover.

CONCLUSIONS:

We demonstrate the feasibility of awake upper-body ECMO to bridge PAH patients to recovery or lung transplantation when optimal medical therapy fails. An upper-body configuration facilitates physical therapy, preventing deconditioning and optimizing transplant candidacy.

CLINICAL IMPLICATIONS:

Awake ECMO should be considered in cases of refractory PAH in whom recovery or transplantation is anticipated.DISCLOSURE: Daniel Brodie: Grant monies (from industry related sources): Daniel Brodie has research support from Maquet Cardiovascular including travel awards to research meetingsMatthew Bacchetta: Grant monies (from industry related sources): Matthew Bacchetta has research support from Maquet Cardiovascular including travel awards to research meetingsThe following authors have nothing to disclose: Darryl Abrams, Erika Rosenzweig, Cara Agerstrand, Joshua SonettNo Product/Research Disclosure InformationColumbia University Medical Center, New York, NY.

SESSION TYPE: Heart FailurePRESENTED ON: Sunday, October 21, 2012 at 10:30 AM - 11:45 AM

PURPOSE:

Atrial septal defects (ASD) and patent foramen ovale (PFO) are the most common adult congenital heart disorders. A common indication for ASD closure is right ventricular enlargement. We report our experience with a cohort of patients who presented with unexplained shortness of breath and hypoxia.

METHODS:

We performed retrospective chart review of patients with ASD/PFO who presented with hypoxia or dyspnea on exertion and were referred to our pulmonary hypertension clinic from January 2010 to February 2012.

RESULTS:

We identified 15 patients (12 female), ages 15-78 (female mean age, 56; male mean age, 67). Seven patients (46.7%) had a tunnel PFO (four with right-to-left shunting, three with bidirectional shunting). Eight patients (53.3%) had ASD (one sinus venosus, seven secundum). Of the seven patients with a secundum ASD, five had left-to-right shunting and two had bidirectional shunting. Baseline functional status was NYHA Class IV in three patients (20%), Class III in 11 (73.3%) and Class II in one (6.7%). Five patients (33.3%) required continuous-flow oxygen. Five patients (33.3%) had severe pulmonary hypertension at diagnosis. Fourteen patients (93.3%) underwent percutaneous closure; one (6.7%) underwent surgical closure. All patients had improvement in NYHA class. Four out of five (80%) oxygen-dependant patients were taken off oxygen, and one had a decrease in oxygen requirement. All five pulmonary hypertension patients were weaned off associated medications. Follow-up echocardiography showed no evidence of residual shunts. One patient with syncope had complete resolution of her symptoms.

CONCLUSIONS:

There is a subset of patients with atrial level shunts who present with disproportionate hypoxia or shortness of breath. In these patients, careful evaluation should include a transesophageal echocardiography with saline contrast imaging and exercise hemodynamics. In our cohort, closure of the atrial shunt led to absolute resolution of their signs and symptoms, improvement in the NYHA class, decrease in oxygen requirements and resolution of pulmonary hypertension.

CLINICAL IMPLICATIONS:

Patients with atrial shunts and dyspnea require additional evaluations as they frequently can be cured by closure.DISCLOSURE: The following authors have nothing to disclose: Krishna Nagendran, Bijoy K. Khandheria, Tanvir Bajwa, Suhail Allaqaband, Mary E. Wenzel, A. Jamil Tajik, Dianne ZwickeNo Product/Research Disclosure InformationAurora Cardiovascular Services, Aurora Sinai/Aurora St. Luke's Medical Centers, University of Wisconsin School of Medicine and Public Health, Milwaukee, WI.

Levoatrial cardinal vein is a rare congenital anomaly of the systemic veins. It is commonly associated with left-sided obstructive conditions such as aortic atresia, mitral atresia, and cor triatriatum. We report the case of a 14-year-old boy who was undergoing surgery for correction of a secundum atrial septal defect. Intraoperatively, we discovered that he had a levoatrial cardinal vein and no brachiocephalic vein. However, collateral vessels provided adequate flow to the right atrium, and the patient's left-sided venous pressure was not excessive, so we ligated the levoatrial cardinal vein and directly repaired the septal defect. Postoperatively, the left venous drainage was satisfactory and the patient was asymptomatic. In addition to our patient's case, we discuss the embryology, diagnosis, and treatment of levoatrial cardinal vein.

Depression is often not optimally treated during pregnancy, partially because of conflicting data regarding antidepressant medication risk. This meta-analysis was conducted to determine whether antenatal antidepressant exposure is associated with congenital malformations and to assess the effect of known methodological limitations.EMBASE, CINAHL, PsycINFO, and MEDLINE were searched from their start dates to June 2010. Keywords of various combinations were used, including, but not limited to depressive/mood disorder, pregnancy, antidepressant drug/agent, congenital malformation, and cardiac malformation.English language studies reporting congenital malformations associated with antidepressants were included. Of 3,074 abstracts reviewed, 735 studies were retrieved and 27 studies were included.Two reviewers working independently assessed article quality. Data on use of any antidepressant, including fluoxetine and paroxetine specifically, were extracted. Outcomes included congenital malformations, major congenital malformations, cardiovascular defects, septal heart defects (ventral septal defects and atrial septal defects), and ventral septal defects only.Nineteen studies were above quality threshold and make up the primary meta-analyses. Pooled relative risks (RRs) were derived by using random-effects methods. Antidepressant exposure was not associated with congenital malformations (RR = 0.93; 95% CI, 0.85-1.02; P = .113) or major malformations (RR = 1.07; 95% CI, 0.99-1.17; P = .095). However, increased risk for cardiovascular malformations (RR = 1.36; 95% CI, 1.08-1.71; P = .008) and septal heart defects (RR = 1.40; 95% CI, 1.10-1.77; P = .005) were found; the RR for ventral septal defects was similar to septal defects, although not significant (RR = 1.54; 95% CI, 0.71-3.33; P = .274). Pooled effects were significant for paroxetine and cardiovascular malformations (RR = 1.43; 95% CI, 1.08-1.88; P = .012). These results are contrasted with those addressing methodological limitations but are typically consistent.Overall, antidepressants do not appear to be associated with an increased risk of congenital malformations, but statistical significance was found for cardiovascular malformations. Results were robust in several sensitivity analyses. Given that the RRs are marginal, they may be the result of uncontrolled confounders. Although the RRs were statistically significant, none reached clinically significant levels.

A full-term male neonate presented with cyanosis upon delivery and was subsequently diagnosed with d-transposition of the great arteries, ventricular septal defect, and restrictive atrial septal defect. Following initiation of intravenous prostaglandins and balloon atrial septostomy, an arterial switch operation was performed on day 3 of life. The postoperative course was complicated by intractable ventricular tachycardia that was refractory to lidocaine, amiodarone, esmolol, fosphenytoin, and mexiletine drug therapy. Ventricular tachycardia was suppressed with overdrive atrial pacing but recurred upon discontinuation. Seven weeks postoperatively, radiofrequency catheter ablation was performed due to hemodynamically compromising persistent ventricular tachycardia refractory to medical therapy. The ventricular tachycardia was localized to the inferior-lateral right ventricular outlet septum. The procedure was successful without complications or recurrence. Antiarrhythmics were discontinued after the ablation procedure. Seven days after the ablation, a different, slower fascicular rhythm was noted to compete with the infant's sinus rhythm. This was consistent with the preablation amiodarone having reached subtherapeutic levels given its very long half-life. The patient was restarted on oral beta blockers and amiodarone. The patient was subsequently discharged home in predominantly sinus rhythm with intermittent fascicular rhythm.

The purpose of this review/editorial is to discuss how and when to treat the most common cyanotic congenital heart defects (CHDs); the discussion of acyanotic heart defects was presented in a previous editorial. By and large, the indications and timing of intervention are decided by the severity of the lesion. While some patients with acyanotic CHD may not require surgical or transcatheter intervention because of spontaneous resolution of the defect or mildness of the defect, the majority of cyanotic CHD will require intervention, mostly surgical. Total surgical correction is the treatment of choice for tetralogy of Fallot patients although some patients may need to be palliated initially by performing a modified Blalock-Taussig shunt. For transposition of the great arteries, arterial switch (Jatene) procedure is the treatment of choice, although Rastelli procedure is required for patients who have associated ventricular septal defect (VSD) and pulmonary stenosis (PS). Some of these babies may require Prostaglandin E1 infusion and/or balloon atrial septostomy prior to corrective surgery. In tricuspid atresia patients, most babies require palliation at presentation either with a modified Blalock-Taussig shunt or pulmonary artery banding followed later by staged Fontan (bidirectional Glenn followed later by extracardiac conduit Fontan conversion usually with fenestration). Truncus arteriosus babies are treated by closure of VSD along with right ventricle to pulmonary artery conduit; palliative banding of the pulmonary artery is no longer recommended. Total anomalous pulmonary venous connection babies require anastomosis of the common pulmonary vein with the left atrium at presentation. Other defects should also be addressed by staged correction or complete repair depending upon the anatomy/physiology. Feasibility, safety and effectiveness of treatment of cyanotic CHD with currently available medical, transcatheter and surgical methods are well established and should be performed at an appropriate age in order to prevent damage to cardiovascular structures.

Holt-Oram syndrome is a rare inherited disorder that causes abnormalities of the hands, arms, and the heart. Most commonly, there are defects in the carpal bones of the wrist and in the bones of the thumb along with cardiac defects such as atrial or ventricular septal defects. We report a case of Holt-Oram syndrome with a rare association of double outlet right ventricle.

An alternative technique for minimally invasive aortic valve replacement and atrial septal defect repair is described. After a 5-cm skin incision, a key-lock type sternotomy is made. Excellent exposure of the right atrium and aortic valve was achieved. The configuration of the mini-sternotomy (or the lock) limits the movement of the sternal surfaces (or the key) on the lateral and craniocaudal directions.