Pulmonary Outflow Tract Obstruction Research Articles

Clinical implications of dextrocardia based on four visceroatrial situs studies

BackgroundPrevious studies on congenital heart diseases (CHD) associated with dextrocardia were based on selective patient databases and did not reflect the full spectrum of dextrocardia in the general population. Additionally, these studies had complex classification and presentation. Nor did these studies elaborate on the distribution of the associated CHD's complexity, the various segmental connections, and associated CHD among the four visceroatrial situs. MethodsWe retrospectively reviewed the medical records of 211 children with primary dextrocardia. We used a segmental approach to diagnose CHD. We then analyzed and compared the distribution of the above-mentioned issues among the four visceroatrial situs. ResultsDextrocardia occurred most commonly with situs inversus (52.6%), followed by situs solitus (28.4%), asplenia (17.1%), and polysplenia (1.9%). Although some patients had a structurally normal heart (22.7%) or they were associated with simple CHD (17.5%), most patients had complex CHD (59.7%) consisting of a single ventricle (34.6%) or conotruncal anomaly (25.1%) (double-outlet right ventricle [7.6%], corrected transposition of the great arteries [6.2%], complete transposition of the great arteries [5.7%], tetralogy of Fallot [4.7%], etc.). Situs inversus or polysplenia had a higher prevalence of a structurally normal heart or associated with simple CHD, two patent atrioventricular (AV) valves connections, and biventricular AV connections. Situs solitus or asplenia had a higher prevalence of associated complex CHD, common AV valve connection, univentricular AV connection, pulmonary outflow tract obstruction, and anomalous pulmonary venous drainage. ConclusionOur study finds that situs inversus is the most common visceroatrial situs in dextrocardia. Although some patients had a structurally normal heart or were associated with simple CHD, most patients have associated complex CHD consisting of a single ventricle or conotruncal anomaly. Dextrocardia is associated with a higher incidence of complex CHD in situs solitus and asplenia groups than in situs inversus and polysplenia groups.

Pulmonary hypertension-related dynamic left ventricular outflow tract obstruction.

Pulmonary hypertension-related dynamic left ventricular outflow tract obstruction.

A BREATHTAKING CENTRAL LINE

SESSION TITLE: Medical Student/Resident Imaging Posters SESSION TYPE: Med Student/Res Case Rep Postr PRESENTED ON: October 18-21, 2020 INTRODUCTION: Central venous catheters (CVC) are used commonly in critically ill patients. It is common practice done daily in the Intensive Care Units. Complications such as arrhythmias, arterial puncture, pneumothorax, thrombosis and rarely, but most lethal, air emboli can occur. We present a case of systemic air embolism after removal of CVC, where direct visualization of right atrium with point of care ultrasound (POCUS) showed presence of air bubbles. CASE PRESENTATION: 53-year-old female with history of IV drug abuse and COPD was admitted for sepsis, septic arthritis and skin abscesses, for which she required a prolonged course of IV antibiotics. Right internal jugular CVC was placed due to contraindication of peripheral IV access. CVC was no longer needed and removed, within a few seconds, patient became restless, tachycardic and tachypneic, with significant accessory muscles use. CXR was negative for pneumothorax or other abnormalities. Patient was placed on non-rebreather mask with 100% oxygen, only achieving 78% saturation. Initial ABG showed acute hypoxemic hypercarbic respiratory failure. It came to our knowledge that removal was done in upright position; air emboli was suspected and confirmed on POCUS (Figure 1). She was emergently intubated, mechanically ventilated on 100% FiO2 and placed in Trendelenburg position. Follow up echocardiogram showed no evidence of air emboli. No neurologic deficits were identified, patient was weaned off high FiO2 and extubated. DISCUSSION: Air emboli can occur during insertion, while within patient or during removal. Air embolism is managed mainly with supportive care. Incidence can be as low as 0.13%, despite using optimal positioning and placement techniques. True incidence is uncertain due to absorption of air prior to confirmation. The presence of air in vasculature results in acute hypoxemia and hypercapnia. If suspected, treatment includes stopping air entry into the system, aspiration of air from the right ventricle if possible, placing in Trendelenburg and left lateral decubitus position. This positioning allows for entrapped air in the heart to stabilize within the apex of the ventricle and relieve obstruction of pulmonary outflow tract. If severe, cardiac ischemia, arrhythmias, hypotension, and cardiac arrest can ensue. Rapid recognition and treatment can prevent this. CONCLUSIONS: Air embolism is a fatal complication during insertion/removal of CVC. Clinical manifestations range from none to cardiovascular collapse and death. Therefore education on proper care and technique is essential. These include explaining the procedure to the patient, laying them supine in Trendelenburg position and instructing to perform Valsalva maneuver on removal. Utilization of POCUS may facilitate early diagnosis and improve outcomes. Physicians and nursing staff should be aware of this iatrogenic complication and be able to identify it in a timely fashion. Reference #1: Marek A. Mirski, Abhijit Vijay Lele, Lunei Fitzsimmons, Thomas J. K. Toung; Diagnosis and Treatment of Vascular Air Embolism. Anesthesiology2007;106(1):164-177. doi: https://doi.org/. Reference #2: J Emerg Trauma Shock. 2009 Sep-Dec; 2(3): 180–185. doi: 10.4103/0974-2700.55330 Reference #3: Khan H, Zaidi A Paradoxical air embolism following central venous catheter removal Case Reports 2013;2013:bcr2013200630. DISCLOSURES: No relevant relationships by Lakshmi Alahari, source=Web Response No relevant relationships by Ammar Aliuddin, source=Web Response No relevant relationships by Gerardo Catalasan, source=Web Response No relevant relationships by Gabriela Orellana, source=Web Response

Comparison of morphologic profile of congenital heart defects associated with right isomerism and left isomerism in Western Indian population

Context: Heterotaxy syndrome is a disorder that involves abnormal lateralization of the abdominal viscera, thoracic organs, and cardiac atria. The objective was to compare the frequency of morphologic profile of congenital heart defects associated with right isomerism and left isomerism in the Western Indian population. Materials and Methods: This cross-sectional observational study was conducted by the Department of Pediatric Cardiology, U N Mehta Institute of Cardiology and Research Centre, Ahmedabad, Gujarat, India, from January 2016 to April 2018. All children presenting to the hospital with suspected congenital heart defect were included in the study. Echocardiography-based sequential segmental analysis was used; various cardiac defects were noted for patients with right isomerism (RAI) and left isomerism (LAI). Results: Occurrence of heterotaxy in our screening population is 0.12%. A total of 93 children had atrial heterotaxy (M: F; 1.06:1), with 65 (69.89%) having RAI and 28 (30.11%) having LAI. Most common lesions associated with RAI included complete atrioventricular septal defect (n = 43, 66.2%), and pulmonary outflow tract obstruction (n = 35, 53.84%). LAI was associated with atrial septal defect (n = 24, 85.7%) ventricular septal defect (n = 23, 82.1%). Conclusion: Heterotaxy encompasses a wide spectrum of congenital cardiac defects. The frequency of various defects associated with RAI and LAI in Western India is compared here to those as reported in the Western world.

Surgical Treatment for Double Outlet Right Ventricle With Pulmonary Outflow Tract Obstruction.

Double outlet right ventricle (DORV) is a conotruncal anomaly that is a defining element of many types of complex congenital heart disease. Because of a big variety of pathology, there are still some controversies with respect to the definition, classification, and surgical treatment. We report our experience with surgical treatment for DORV (as defined by the "90% rule") with pulmonary outflow tract obstruction (POTO). From July 2005 to July 2015, 90 patients underwent surgical treatment of DORV with POTO at the First Hospital of Tsinghua University. There were 55 males and 35 females whose age varies from 3 months to 36 years (mean age 7.1 ± 9.0 years old), and body weights ranged from 5 to 63 kg (mean weight 20.4 ± 16.6 kg). Besides DORV, ventricular septal defect, and POTO, this group of patients includes some with additional associated cardiac abnormalities. Fourteen patients (15.6%) died. The main cause of death was low cardiac output syndrome. The DORV is usually associated with a variety of cardiac abnormalities and POTO is a common defining feature. Acceptable surgical results can be achieved by individualized surgical treatment of most patients. Some patients may require reoperation, and a close follow-up is needed.

Subpulmonary Obstruction from Aneurysmal Ventricular Septum in a Child with Dextrocardia and Congenitally Corrected Transposition of the Great Arteries

A 3-year-old boy underwent evaluation for dextrocardia. Echocardiograms showed features of corrected transposition physiology, a perimembranous ventricular septal defect (VSD) (Fig. 1), and aneurysmal tissue beneath the pulmonary valve that caused severe subpulmonary stenosis (Figs. 2–4). Eighteen months after VSD closure and resection of the aneurysmal tissue, the patient was asymptomatic with only mild residual pulmonary outflow tract obstruction. Fig. 1. Two-dimensional echocardiograms A) in apical 4-chamber view from the right chest and B) in color-flow Doppler mode show a moderate-to-large ventricular septal defect (VSD). Fig. 2. Echocardiograms (subcostal 4-chamber views). A) In the morphologic left ventricle (MLV), aneurysmal tissue (arrow) protrudes into the pulmonary outflow tract. B) Color-flow Doppler mode reveals turbulent flow (arrow) in the pulmonary outflow tract. Fig. 3. A) Continuous-wave Doppler echocardiographic recording across the pulmonary outflow tract shows a peak velocity >5 m/s, suggesting severe obstruction. B) Angiogram (60° left anterior oblique view) from the morphologic left ventricle (MLV) ... Fig. 4. Cineangiographic image from the A) left lateral view reveals the morphologic left ventricle (MLV) and the pulmonary outflow tract obstruction from aneurysmal tissue (arrows). B) The 60° left anterior oblique view reveals a severely narrowed right ...

Non-invasive estimation of pulmonary outflow tract obstruction: A comparative study of cardiovascular phase contrast magnetic resonance and Doppler echocardiography versus cardiac catheterization

AimTo compare estimated pressure gradients from routine follow-up cardiovascular phase-contrast magnetic resonance (PC-MR) with those from Doppler echocardiography and invasive catheterization in patients with congenital heart disease (CHD) and pulmonary outflow tract obstruction. MethodsIn 75 patients with pulmonary outflow tract obstruction maximal and mean PC-MR gradients were compared to maximal and mean Doppler gradients. Additionally, in a subgroup of 31 patients maximal and mean PC-MR and Doppler pressure gradients were compared to catheter peak-to-peak pressure gradients (PPG). ResultsMaximal and mean PC-MR gradients underestimated pulmonary outflow tract obstruction as compared to Doppler (max gradient: bias=+8.4mmHg (+47.6%), r=0.89, p<0.001; mean gradient: +4.3mmHg (+49.0%), r=0.88, p<0.001). However, in comparison to catheter PPG, maximal PC-MR gradients (bias=+1.8mmHg (+8.8%), r=0.90, p=0.14) and mean Doppler gradients (bias=−2.3mmHg (−11.2%), r=0.87, p=0.17) revealed best agreement. Mean PC-MR gradients underestimated (bias=−7.7mmHg (−55.6%), r=0.90, p<0.001) while maximal Doppler gradients systematically overestimated catheter PPG (bias=+13.9mmHg (+56.5%), r=0.88, p<0.001). ConclusionsEstimated maximal PC-MR pressure gradients from routine CHD follow-up agree well with invasively assessed peak-to-peak pressure gradients. Estimated maximal Doppler pressure gradients tend to overestimate, while Doppler mean gradients agree better with catheter PPG. Therefore, our data provide reasonable arguments to either apply maximal PC-MR gradients or mean Doppler gradients to non-invasively evaluate the severity of pulmonary outflow tract obstruction in the follow-up of CHD.

Value of a novel categorization of congenital double-outlet right ventricle on guiding the choice of surgical approaches

To explore the feasibility and value of a new categorization of double outlet right ventricular (DORV) on guiding the optimal choices of surgical approaches. Five hundred and twenty one DORV patients diagnosed by echocardiography, angiocardiography and CT at Fuwai Hospital from May 2003 to September 2014 were enrolled in this retrospective study. Congenital DORV was categorized according to three basic factors as follows: the positional relationships of great arteries (normal relation or abnormal relation), the relationships of the ventricular septal defect (VSD) to the great arteries (committed VSD or remote VSD), the presence or absence of pulmonary outflow tract obstruction (POTO). Eight types of DORV were established: type I (normal relation, committed VSD, without POTO), type II (normal relation, committed VSD, POTO), type III (normal relation, remote VSD, without POTO), type IV (normal relation, remote VSD, POTO), type V (abnormal relation, committed VSD, without POTO), type VI (abnormal relation, committed VSD, POTO), type VII (abnormal relation, remote VSD, without POTO), type VIII (abnormal relation, remote VSD, POTO). Feasibility of this classification and the value of this classification on guiding the choice of surgical approaches were analyzed. Among the five hundred and twenty one patients, there were 90 patients (17.3%) with type I DORV, 94 patients (18.0%) with type II, 33 patients (6.3%) with type III, 34 patients (6.5%) with type IV, 64 patients (12.3%) with type V, 61 patients (11.7%) with type VI, 33 patients (6.3%) with type VII, 112 patients (21.5%) with type VIII. Thus, all patients could be typed by this classification method. The echocardiography diagnosis was consistent with the intra-operative and or cardiac catheterization/CT findings. Excluding the contraindications of bi-ventricular repair, different surgical approaches were performed in every subtype of DORV according the classification, which indicated that this novel categorization could accurately guide the clinic managements. This novel DORV categorization can accurately diagnose DORV lesions, and guide the clinic therapy choice.

Twenty-five years' experience of modified Lecompte procedure for the anomalies of ventriculoarterial connection with ventricular septal defect and pulmonary stenosis

To overcome the drawbacks of the Rastelli operation, the modified Lecompte procedure creates a connection from the left ventricle to the aorta with greater freedom from residual obstruction due to the resection of the outlet septum and avoids the implantation of an extracardiac valved conduit. We evaluated the effectiveness of this technique with analysis of our 25-year long-term results. We reviewed the records of 50 patients who underwent the modified Lecompte procedure during the past 25 years. The median age at operation was 1.95 years (range, 0.30-12.48 years). The diagnoses involved anomalies of the ventriculoarterial connection with ventricular septal defect and pulmonary outflow tract obstruction, such as transposition of the great arteries, double outlet right ventricle, and double outlet left ventricle. There were 2 early deaths (4.0%). During a mean follow-up of 14.2 ± 7.9 years (range, 0.2-25 years), there were 4 late deaths. Actuarial survival was 87.3% ± 4.9% at 25 years. The freedom from arrhythmia and reoperation at 25 years was 87.7% ± 6.2% and 25.2% ± 9.4%, respectively. The freedom from reoperation for left ventricular outflow tract obstruction and right ventricular outflow tract obstruction at 25 years was 88.5% ± 5.4% and 49.6% ± 9.0%, respectively. At last follow-up, 43 survivors (97.7%) are in New York Heart Association class I. The modified Lecompte procedure has excellent long-term results for treating anomalies of ventriculoarterial connection with ventricular septal defect and pulmonary outflow tract obstruction. Early repair is possible with low mortality and morbidity in terms of arrhythmia, reoperation for right or left ventricular outflow tract obstruction, and functional class.

Pulmonary artery blood flow patterns in fetuses with pulmonary outflow tract obstruction

Fetuses with pulmonary outflow tract obstruction (POTO) have altered blood flow to the pulmonary vasculature. We sought to determine whether pulmonary vascular impedance, as assessed by the pulsatility index (PI), is different in fetuses with POTO compared with normal controls. Branch pulmonary artery PI was evaluated in age-matched normal control fetuses (n=22) and 20 POTO fetuses (pulmonary stenosis n=15, pulmonary atresia n=5). Pulsed-wave Doppler was performed in the proximal (PA1), mid (PA2) and distal (PA3) branch pulmonary artery. The direction of flow in the ductus arteriosus was noted. The study and control groups were compared with Student's t-test and ANOVA. A linear mixed model evaluated the relationship between PI and ductus arteriosus flow patterns. There was no difference in PI between control, pulmonary stenosis and pulmonary atresia subjects at PA1 and PA2; however, there was a significant difference at PA3. Subjects with pulmonary atresia had a lower PI at PA3 than did controls (P=0.003) and pulmonary stenosis subjects (P=0.003). Subjects with retrograde flow in the ductus arteriosus had lower PIs in PA2 and PA3 than did those with antegrade flow (P=0.01 and 0.005, respectively). The PI in PA3 was lower in fetuses that required prostaglandin postnatally than in those that did not (P=0.008). Fetuses with pulmonary atresia or severe pulmonary stenosis with retrograde flow in the ductus arteriosus have decreased PI in the distal pulmonary vasculature. Our findings indicate the capacity of the fetal pulmonary vasculature to vasodilate in response to anatomical obstruction of flow.

Severe left heart obstruction with retrograde arch flow influences fetal cerebral and placental blood flow

Decreased middle cerebral artery (MCA) pulsatility index (PI) is a marker of fetal brain-sparing in placental insufficiency and it is also found in fetuses with severe congenital heart disease. This study sought to explore the impact of anatomical subtypes in fetal heart disease on MCA-PI and head growth. We retrospectively reviewed fetal echocardiograms of pregnancies complicated by fetal hypoplastic left heart syndrome (HLHS; n = 42) with and without anatomic coarctation (n = 28 and n = 10, respectively), isolated severe aortic coarctation (n = 21), D-transposition of the great arteries (TGA; n = 11) and pulmonary outflow tract obstruction without forward flow across the pulmonary valve (POTO; n = 15), comparing observations with gestational age-matched controls (n = 89). No fetus had major extracardiac pathology or aneuploidy. MCA and umbilical artery (UA) PI, the cerebral placental ratio (CPR = MCA-PI/ UA-PI) and neonatal head circumference were obtained and expressed as Z-scores. Lower MCA-PI, higher UA-PI and lower CPR were observed in fetal HLHS and isolated coarctation with reversed arch flow (n = 6) (P < 0.001) but not TGA, POTO or isolated coarctation with antegrade arch flow (n = 15) compared with controls. No difference was found between HLHS with anatomical coarctation and those without; however, MCA-PI correlated positively with neonatal head circumference in HLHS with reversed distal arch flow (r = 0.33, P < 0.05). Severe left heart obstruction with reversed aortic arch flow is associated with altered fetal cerebral blood flow, and in these conditions, MCA-PI positively correlates with head growth. Anatomical arch obstruction itself may not be a contributing factor to altered MCA flow in fetal HLHS.

“V-PLASTY”: a novel technique to reconstruct pulmonary valvular and annular stenosis in patients with right ventricular outflow tract obstruction

BackgroundThe goal of repair of right ventricular outflow tract obstruction with or without Tetralogy of Fallot (TOF) is to eliminate valvular and/or subvalvular obstruction. However, this operation has a high risk of late complication of pulmonary insufficiency. In this study, we aimed to present early period results of our new technique that we call “V-Plasty” developed to prevent pulmonary insufficiency after pulmonary valve reconstruction in selected patients.MethodsBetween January 2006 and January 2010, we performed V-plasty for pulmonary valve reconstruction in 10 patients. Eight patients (5 males, 3 females) had TOF and 2 patients (1 male, 1 female) had atrial septal defect concomitant with pulmonary valve stenosis. Patient selection for V-plasty reconstruction was made due to the pulmonary valve anatomy and degree of stenosis. The mean follow-up time was 55.7 ± 16.2 months (ranging from 32 to 80 months).ResultsFunctional capacity of the patients improved immediately after the surgery. There were no mortality and re-operation in follow-up period. Patients were followed up with echocardiography one week after the operation, at 1st, 6th, 12th months and annually. There was no pulmonary insufficiency.ConclusionsOperative correction of the pulmonary outflow tract obstruction with or without TOF, frequently requires transannular enlargement because of the infundibular and/or annular-valvular obstruction. This conventional technique is usually a reason for late pulmonary insufficiency. In our study, we have not seen pulmonary insufficiency in early term follow-up period. Our early term results are encouraging, but long term follow-up results are needed with large case series.

Pulmonary outflow tract obstruction in fetuses with complex congenital heart disease: predicting the need for neonatal intervention

To identify prenatal echocardiographic markers that could predict the need for neonatal intervention in fetuses with right ventricular outflow tract obstruction. This was a retrospective study of 52 fetuses with right ventricular outflow tract obstruction. Echocardiograms were evaluated for fetuses with either two-ventricle anatomy with a large ventricular septal defect or single-ventricle anatomy. Fetuses with pulmonary atresia were excluded. Parameters were compared between groups that did and did not require an intervention at age < 30 days. Fifty-two fetuses were studied; 20 (38%) underwent neonatal intervention and 32 (62%) did not. The most common diagnosis was tetralogy of Fallot (n = 32). Fetuses with two ventricles that required an intervention had lower pulmonary valve diameter Z-score (PV-Z-score) (-4.8 ± 2.1 vs. -2.6 ± 1.1; P = 0.0002) and lower pulmonary valve to aortic valve annular diameter ratio (PV/AoV) (0.53 ± 0.15 vs. 0.66 ± 0.1; P = 0.003). Using a PV/AoV ratio of < 0.6 or a PV-Z-score of < -3 at final echocardiographic examination was highly sensitive (92%) but poorly specific (50%), whereas classifying direction of flow in the ductus arteriosus as either normal (all pulmonary-to-aorta) or abnormal (aorta-to-pulmonary or bidirectional) was both highly sensitive (100%) and specific (95%) for predicting the need for a neonatal intervention. Parameters for the single-ventricle cohort did not reach statistical significance. Analysis of the pulmonary outflow tract and ductus arteriosus flow in the fetus with complex congenital heart disease can aid in identifying those that will require a neonatal intervention to augment pulmonary blood flow. This has important implications for the planning of delivery strategies.

Congenital heart defects associated with atrial heterotaxy.

To determine the frequency of various congenital cardiac defects associated with atrial heterotaxy and the difference between spectrum of cardiac defects in right atrial isomerism (RAI) and left atrial isomerism (LAI) in these patients. Cross-sectional observational study. Department of Cardiology, The Children's Hospital and Institute of Child Health, Lahore, from July 2006 to June 2011. All children presenting to the hospital with suspected congenital heart defect were included in the study. Echocardiography based sequential segmental analysis was used, taking atrial symmetry, interrupted inferior vena cava (IVC) and juxtaposed aorta to IVC relation into account for identifying atrial heterotaxy. Various segmental defects were noted for RAI and LAI. Children had atrial heterotaxy (M:F; 1.7:1) with 61.6% (n=82) having LAI. Most common lesions associated with RAI included complete atrioventricular (AV) septal defect (n=48, 94.1%), single AV valve (n=45, 88.2%) and pulmonary outflow tract obstruction (n=41, 80.4%). LAI was associated with ventricular septal defect (n=68, 82.9%), atrial septal defect (n=63, 76.8%, mostly secundum variety), and miscellaneous left sided obstructive lesions. Atrial heterotaxy encompasses a wide spectrum of congenital cardiac defects. The frequency of various defects associated with RAI or LAI in local South Asian population of Lahore, Pakistan is similar to those as reported in the Western literature.

The Outcome of Patients With Right Atrial Isomerism is Poor

Right-atrial isomerism (RAI) is a heterotaxy syndrome with disturbances of left-right axis development resulting in complex heart malformations and anomalies of the thoracic and abdominal organs. To study the outcome of RAI, all data from patients diagnosed with this syndrome at Helsinki University Hospital between January 1976 and December of 2010 were reviewed. The outcomes were studied for 32 patients (38 % girls). The overall survival was 22 % at a median follow-up time of 13.8 years (range 0.1-33). Extracardiac malformations, mostly asplenic, occurred in 91 % of patients. Cardiac defects included dextrocardia in 44 % and common atrioventricular valve in 100 % of patients. Ventriculoarterial discordance or double-outlet ventricle was seen in 56 and 44 % of patients, respectively. Total anomalous pulmonary venous drainage occurred in 75 % and partially anomalous venous drainage in 13 % of patients. Pulmonary outflow-tract obstruction was identified in 91 % of patients. Cardiac arrhythmias were noted in nine patients (28 %), two of them with atrioventricular block. Cardiovascular surgery was performed in 71 % patients (N = 25), seven patients were inoperable. Biventricular repair was not possible in any of the patients. During long-term follow-up there was no significant difference between the patients with total, normal, or partially anomalous pulmonary venous drainage (P = 0.5). In conclusion, RAI is one of the most severe forms of congenital cardiac diseases. The prognosis remains poor despite modern surgical techniques. When RAI is identified during pregnancy, prenatal counseling, termination, or planning for prompt cardiac treatment after the birth is necessary.

Right ventricular myxoma causing pulmonary outflow tract obstruction

A patient presented with shortness of breath, lethargy and weight loss. A computerized tomography and echocardiogram showed a mass in the right ventricle nearly obstructing the pulmonary valve during systole and prolapsing into the main pulmonary artery. The mass was completely excised. Histology was that of a typical myxoma.

Noninvasive diagnosis of an adult patient with double-outlet left ventricle

Fig. 1. Transthoracic (TTE) and transesophageal (TEE) echocardiographic images. A. Apical four chamber image in TTE shows tricuspid atresia (arrow) and atrial septal defect. B. Suprasternal image in TTE shows hypoplastic main pulmonary artery and well developed branch pulmonary arteries. C. Transverse plane image in TEE shows malposition of great arteries. The aorta is anterior and right to the pulmonary artery (D-malposition). Large atrial septal defect can be identified as well. D. Longitudinal plane image in TEE shows both great arteries arising from the left ventricle in parallel fashion. There is severe pulmonary outflow tract obstruction and pulmonary conus is present (arrow). PA: pulmonary artery; AO: aorta; and LA: left atrium.

Right ventricular outflow tract obstruction as a confounding factor in the assessment of the impact of pulmonary regurgitation on the right ventricular size and function in patients after repair of tetralogy of fallot

To compare right ventricular (RV) size and function between patients with combined pulmonary regurgitation (PR) plus RV outflow tract (RVOT) obstruction (RVOTO) and patients with isolated PR. Consecutive individuals with significant PR (PR fraction ≥ 20%) after tetralogy of Fallot (TOF) repair who underwent cardiovascular magnetic resonance (CMR) were included. Patients with additional hemodynamic abnormalities (residual ventricular septal defect, extracardiac shunt, and/or more than mild regurgitation at a valve other than the pulmonary valve) were excluded. Significant RVOTO was defined as peak gradient across RVOT ≥ 30 mmHg. Significant differences between patients with combined PR+RVOTO (n = 9) and isolated PR (n = 33) were observed in RV end-diastolic volume (138.6 ± 25.1 vs. 167.0 ± 34.6 mL/m(2) , P = 0.02, respectively), RV end-systolic volume (65.0 ± 9.6 vs. 92.7 ± 26.2 mL/m(2) , P = 0.003), and RV ejection fraction (RVEF) (52.8 ± 3.7 vs. 45.0 ± 6.4%, P = 0.001). Both PR and peak RVOT gradient were independent predictors of RV size. Patients with combined PR+RVOTO had smaller RV volumes and higher RVEF when compared with patients with isolated PR. The confounding effect of RVOTO on RV size and function needs to be considered in CMR studies evaluating patients after TOF repair.

Half rotation of the truncus arteriosus plus arterial switch for transposition of the great arteries with ventricular septal defect and pulmonary outflow tract obstruction

The goal of our study was to report our experience of using half rotation of the truncus arteriosus plus arterial switch, as a modification of the Nikaidoh procedure, for anatomic repair of transposition of the great arteries (TGA) with ventricular septal defect (VSD) and pulmonary outflow tract obstruction (pulmonary stenosis (PS)). From December 2006 to December 2009, 11 patients (six male, five female) underwent half rotation of the truncus arteriosus plus arterial switch for repair of TGA, VSD, and PS or double-outlet right ventricle (DORV) at our heart center. There was no operative death. All patients had relief of left ventricular outflow tract obstruction (LVOTO) as determined by direct pressure measurements intraoperatively. Echocardiography was performed for all patients intraoperatively and before discharge. Two patients had mild aortic regurgitation and two patients had mild pulmonary regurgitation. For a median follow-up of 15 months (range 6-42 months), all patients were alive. Echocardiography was performed at 3, 6, 12, 24 and 36 postoperative months. All patients have normal ventricular function. No evidence of LVOTO was founded in all patients. Two patients had mild aortic regurgitation and three patients had mild to medium pulmonary regurgitation. Half rotation of the truncus arteriosus plus arterial switch, as a modification of the Nikaidoh procedure, is a good alternative treatment for complete anatomic repair for TGA, VSD, and PS or DORV with malposition of great arteries and PS. The technique might allow to reconstruct biventricular outflow tract, preserve competence and growth potential of the pulmonary root with valves and decrease the probability of reoperation. Its long-term benefits need to be evaluated with a large number of patients and longer follow-up.

Prenatal diagnosis of tricuspid atresia: intrauterine course and outcome

To evaluate the intrauterine course and outcome of tricuspid atresia detected in the fetus. This was a retrospective review of all confirmed cases of tricuspid atresia detected prenatally between 1998 and 2006 in three tertiary referral centers in Germany. Fifty-four cases of tricuspid atresia were detected prenatally during the study period and confirmed postnatally: 28 (51.9%) cases had a concordant ventriculoarterial connection of which 14 also had pulmonary outflow obstruction; 25 (46.3%) cases had a discordant ventriculoarterial connection of which 14 also had aortic outflow obstruction, six had pulmonary outflow tract obstruction and two had other associated intracardiac anomalies; and one (1.9%) had a common arterial trunk. The peak velocity index for veins in the ductus venosus was significantly elevated in 19 of the 37 (51.4%) cases assessed; however, this finding did not correlate with adverse intrauterine outcome. There were associated extracardiac anomalies in 12 cases: five with chromosomal anomalies, two with VACTERL association, one with unilateral renal agenesis, one with hypospadia, one with hydrothorax, one with megacystis and one with agenesis of the ductus venosus. Seventeen of the 54 (31.5%) cases underwent termination of pregnancy, two (3.7%) died in utero, two (3.7%) died in infancy and 33 (61.1%) children survived with a median follow-up of 26 (range, 12-120) months. Prenatal echocardiography correctly anticipated the postnatal course and the need for neonatal intervention in 29/35 (82.9%) continued pregnancies; in the remaining six (17.1%) cases the right outflow tract obstruction had been underestimated. Tricuspid atresia and the frequently associated intracardiac anomalies can be diagnosed in the fetus with considerable accuracy. A thorough search for extracardiac malformations should be performed in order to rule out chromosomal anomalies and multiple malformation syndromes. Elevated pulsatility in the ductus venosus does not indicate cardiac failure. The short-term overall survival in continued pregnancies in our study exceeded 89%, with the greatest rate of loss being in the first year of postnatal life.