Partial Anomalous Venous Return Research Articles

Free-breathing high resolution modified Dixon steady-state angiography with compressed sensing for the assessment of the thoracic vasculature in pediatric patients with congenital heart disease

BackgroundCardiovascular magnetic resonance angiography (CMRA) is a non-invasive imaging modality of choice in pediatric patients with congenital heart disease (CHD). This study was aimed to evaluate the diagnostic utility of a respiratory- and electrocardiogram-gated steady-state CMRA with modified Dixon (mDixon) fat suppression technique and compressed sensing in comparison to standard first-pass CMRA in pediatric patients with CHD at 3 T.MethodsIn this retrospective single center study, pediatric CHD patients who underwent CMR with first-pass CMRA followed by mDixon steady-state CMRA at 3 T were analyzed. Image quality using a Likert scale from 5 (excellent) to 1 (non-diagnostic) and quality of fat suppression were assessed in consensus by two readers. Blood-to-tissue contrast and quantitative measurements of the thoracic vasculature were assessed separately by two readers. CMRA images were reevaluated by two readers for additional findings, which could be identified only on either one of the CMRA types. Paired Student t test, Wilcoxon test, and intraclass correlation coefficients (ICCs) were used for statistical analysis.Results32 patients with CHD (3.3 ± 1.7 years, 13 female) were included. Overall image quality of steady-state mDixon CMRA was higher compared to first-pass CMRA (4.5 ± 0.5 vs. 3.3 ± 0.5; P < 0.001). Blood-to-tissue contrast ratio of steady-state mDixon CMRA was comparable to first-pass CMRA (7.85 ± 4.75 vs. 6.35 ± 2.23; P = 0.133). Fat suppression of steady-state mDixon CMRA was perfect in 30/32 (94%) cases. Vessel diameters were greater in first-pass CMRA compared to steady-state mDixon CMRA with the greatest differences at the level of pulmonary arteries and veins (e.g., right pulmonary artery for reader 1: 10.4 ± 2.4 vs. 9.9 ± 2.3 mm, P < 0.001). Interobserver agreement was higher for steady-state mDixon CMRA for all measurements compared to first-pass CMRA (ICCs > 0.92). In 9/32 (28%) patients, 10 additional findings were identified on mDixon steady-state CMRA (e.g., partial anomalous venous return, abnormalities of coronary arteries, subclavian artery stenosis), which were not depicted using first-pass CMRA.ConclusionsSteady-state mDixon CMRA offers a robust fat suppression, a high image quality, and diagnostic utility for the assessment of the thoracic vasculature in pediatric CHD patients.

PARTIAL ANOMALOUS PULMONARY VENOUS CONNECTION: A RARE CAUSE OF PULMONARY HYPERTENSION

SESSION TITLE: Pulmonary Vascular Disease SESSION TYPE: Fellow Case Report Posters PRESENTED ON: 10/09/2018 01:15 PM - 02:15 PM INTRODUCTION: Partial anomalous pulmonary venous connection (PAPVC) is an uncommon cause of pulmonary hypertension (PH). Isolated partial anomalous pulmonary venous connection is a rare congenital heart disease, with a prevalence of 0.1 to 0.2% in the adult population and incidence is between 0.4 – 0.7%. CASE PRESENTATION: A 69-year-old man with history of mental impairment, hypertension and asthma presented to outpatient PH clinic for evaluation of cardiomegaly and abnormal transthoracic echocardiography (TTE). Review of systems was unremarkable except for easy fatigability. Pertinent examination findings include webbed long neck, micrognatia and loud, split S2 at the base of heart. EKG revealed right bundle branch block and on TTE, the right sided chambers were mildly enlarged with mild to moderate tricuspid regurgitation and an estimated right ventricle systolic pressure (RVSP) of 44.8 mmHg. A right heart catheterization (RHC) confirmed moderate PH with a mean pulmonary artery pressure and wedge pressures measurements: 40, 20 mmHg respectively. Oxygen saturations in the IVC, SVC, RA, RV, PA, PCWP: 76%, 99%, 74%, 83%, 83%, 87% respectively. Left to right shunt was 17.3 L/min. Transesophageal echocardiogram (TEE) did not show an intracardiac shunt on color Doppler and saline contrast. CTA chest confirmed the presence of partial anomalous venous return of the right upper lobe into the superior vena cava (SVC). DISCUSSION: An anomalous pulmonary venous connection occurs in approximately 10–15% of ostium secundum atrial septal defect (ASD) and 85% of patients with sinus venosus ASD. PAPVC is more common from right pulmonary veins compared to left side. Anomalous right-sided pulmonary veins drain into the SVC, right atrium, inferior vena cava, azygos vein, portal vein, or hepatic vein. Anomalous left-sided pulmonary veins drain into the left brachiocephalic vein, coronary sinus, or hemiazygos vein. Patients with PAPVR develop pulmonary arterial hypertension (PAH) from increased venous blood return to the right side of the heart through the anomalous vein. The increase in flow and pressure within the pulmonary vascular bed and the associated shear stress lead to vascular remodeling. Clinical presentation is usually non-specific which includes fatigue, shortness of breath, signs and symptoms of right heart failure, PH. PAPVC is diagnosed by TTE, TEE, pulmonary artery angiography, left and right heart catherization. There are number of surgical corrections for PAPVC based on location of anomaly. An anomalous vein on the left side is implanted directly into the left auricular appendage or the left atrium. Right PAPVC repair consists of complete closure of the septal defect, with redirection of the anomalous pulmonary veins into the left atrium. CONCLUSIONS: Diagnosis of PAPVC is challenging with multimodality imaging and diagnostic workup. Although rare, PAPVC should be kept in differential as a cause of PH. Reference #1: MDCT of Partial Anomalous Pulmonary Venous Return (PAPVR) in Adults Mai-Lan Ho, MD et al Reference #2: Partial Anomalous Pulmonary Venous Return Evaluation of 51 Cases. Filiz SENOCAK et al Reference #3: Partial Anomalous Pulmonary Venous Connection and Pulmonary Arterial Hypertension. Sandeep Sahay et al DISCLOSURES: No relevant relationships by Sameh Aziz, source=Web Response No relevant relationships by Domingo Franco-Palacios, source=Web Response No relevant relationships by Venkateswara Kollipara, source=Web Response

Visual Diagnosis: A 7-year-old Boy with Dyspnea and an Unusual Chest Radiograph

1. Katherine B. Salciccioli, MD* 2. Athar M. Qureshi, MD*,† 3. Hugh D. Allen, MD*,† 1. *Department of Cardiology, Texas Children’s Hospital, Houston, TX 2. †Baylor College of Medicine, Houston, TX A 7-year-old boy presents to his pediatrician with gradually worsening shortness of breath and exercise intolerance over months to years. He was admitted for right-sided pneumonia twice during childhood with seemingly complete recovery each time, but otherwise he has been generally healthy and developmentally appropriate. Current medications include albuterol inhalers taken as needed since early childhood for wheezing during viral illnesses. With the recent worsening of his symptoms, albuterol is again tried without any improvement in his symptoms. He does not have fever, cough, nasal congestion, chest pain, palpitations, vomiting, diarrhea, or weight changes. On physical examination, the patient has normal vital signs. He is small for his age but is following his growth curve appropriately with height in the 15th percentile and weight in the 11th percentile. He has normal findings on head and neck examination. His cardiac examination is notable for a fixed split S2 with no murmurs, rubs, or gallops. He has diminished breath sounds on the right with a soft, intermittent expiratory wheeze and a clear left lung. He has no abdominal organomegaly. Pulses are 2+ and symmetric in all extremities. In addition to his physical examination findings, a chest radiograph (Fig 1) is interpreted as showing dextrocardia, so the patient is referred to the cardiology clinic where echocardiography reveals the diagnosis. Figure 1. Chest radiograph interpreted as dextrocardia, prominent right-sided pulmonary vasculature, and hyperinflation of the left lung. In the cardiology clinic, echocardiography shows the heart located in the right chest, with the cardiac apex pointing leftward, partial anomalous venous return to the inferior vena cava (IVC) (Video 1), and mild right heart dilation (Video 2 …

Partial Anomalous Venous Return of Left Superior Pulmonary Vein in Adult

Drainage of one or more pulmonary veins into rightsided circulation instead of the left atrium is a rarely seen anomaly in adults [1–4]. In the preoperative evaluation of a female patient with esophageal carcinoma, axial (Fig 1A), coronal section (Fig 1B), and three-dimensional reconstruction (Fig 1C) of thorax computed tomography demonstrated abnormal drainage of the left superior pulmonary vein to the left brachiocephalic vein. Because pulmonaryto-systemic shunt size was not large and the patient was asymptomatic, surgical correction was not performed. She underwent esophageal carcinoma surgery.

Levoatriocardinal Vein With Partial Anomalous Venous Return and a Bidirectional Shunt

A 52-year-old woman with a history of systemic lupus erythematosus presented to our institution with nonhealing ulcers on her left lower extremity and toes. She had no cardiac symptoms, with a normal ECG and chest radiograph (Figures 1 and 2). As part of her evaluation she underwent a transesophageal echocardiogram to look for endocarditis, which demonstrated a right-to-left shunt with Valsalva that appeared to originate from the vicinity of the lateral aspect of the left atrium (Movie I in the online-only Data Supplement). There was also a vascular structure seen adjacent to the aorta that typically would represent a remnant of the cardinal vein, such as a persistent left superior vena cava. There were no findings of endocarditis. Figure 1. ECG with normal sinus rhythm. Figure 2. Chest radiograph demonstrates normal heart size and pulmonary vascularity. A magnetic resonance angiogram was performed to further evaluate the origin of the right-to-left shunt and demonstrated a levoatriocardinal vein that drained the left upper lobe …

AJR Teaching File: Dyspnea Following Surgical Repair of Partial Anomalous Venous Return

AJR Teaching File: Dyspnea Following Surgical Repair of Partial Anomalous Venous ReturnBa D. Nguyen1Audio Available | Share

Unusual Case of Anomalous Pulmonary Venous Return With Left Atrial to Systemic Venous Shunt

A 5-year-old boy with an intermittent systolic heart murmur at routine pediatric checkups and an incomplete right bundle-branch block on ECG and with no other past medical history and a normal exercise tolerance presented for echocardiographic evaluation. Echocardiography showed a volume-loaded right ventricle (RV) and right atrium, a patent foramen ovale, and reversal of flow in the right upper pulmonary vein (RUPV). As these views were limited, we proceeded to perform a transesophageal echocardiogram (TEE) and a cardiac catheterization to clarify the anatomy. TEE showed normal connection of all 4 pulmonary veins and significant reversal flow from the left atrium (LA) into the …

Value of transesophageal color Doppler echocardiography for detection of different types of atrial septal defect in adults.

In 121 adults, the value of transthoracic and transesophageal color Doppler echocardiography for detection of different types of atrial septal defect (ASD) or of partial anomalous pulmonary venous return was analyzed. The 121 patients had a total of 129 defects with left-to-right atrial shunting (including eight patients with two types of defects). All of six cases with primum-type ASD were diagnosed correctly by both echocardiographic methods. Ninety-seven patients showed a secundum-type ASD during transesophageal echocardiography: by transthoracic echocardiography, only eight (20%) of the 40 small defects (diameter < 5 mm) were detected as compared with 15 (83%) of the 18 defects with a diameter of 5 to 10 mm and all 39 defects with a diameter > 10 mm. A sinus venosus--type ASD was evident by transesophageal echocardiography in 11 patients, of which only one (9%) was demonstrated by the transthoracic approach. Partial anomalous pulmonary venous return was seen by transesophageal echocardiography in 13 patients but missed in two other patients in whom anomalous pulmonary venous return was subsequently identified by surgery (both with anomalous return of the upper right pulmonary vein into the superior vena cava). By use of the transthoracic technique, partial anomalous venous return was detected in only two cases, both of which had "scimitar syndrome." Compared with transthoracic echocardiography, the transesophageal approach is clearly superior in the detection of small secundum-type ASD, sinus venosus--type ASD, and partial anomalous pulmonary venous return.

CT and MR Imaging of Partial Anomalous Pulmonary Venous Return to the Azygos Vein

Partial anomalous pulmonary venous return to the azygos vein is very unusual, occurring in 0.02% of autopsy examinations. We report the CT, magnetic resonance, and angiographic findings in a 67-year-old man with partial anomalous venous return to the azygos vein.

Intravenous digital subtraction angiography in the assessment of patients with left to right shunts before and after surgical correction

Pre- and postoperative structural changes and pulmonary to systemic flow (QP/QS) ratios were assessed using digital angiography in 34 patients documented to have a left to right shunt at cardiac catheterization. There were 16 men and 18 women whose ages ranged from 4 months to 60 years. The radiographic single mask mode was used for all digital subtraction angiographic studies with a typical radiographic sequence being 80 to 100 kV, 5 to 10 mA/frame at six frames/s for 15 seconds. Renografin-76 was used as a bolus injection at 0.5 to 1.0 ml/kg via an arm vein in most patients. The level of the left to right shunt and any associated anomalies were noted and compared with results from cardiac catheterization. Digital subtraction angiographic flow curves were generated from the pulmonary arteries, and QP/QS ratios were calculated pre- and postoperatively using the gamma variate fit method and compared with the QP/QS ratio from first pass radionuclide studies. A strong correlation between preoperative digital subtraction angiographically derived QP/QS ratio and radionuclide-derived QP/QS ratio was found, with an r value equal to 0.89, p less than 0.0001. Postoperatively, all patients had a QP/QS ratio less than 1.2:1.0 for both digital subtraction angiography and radionuclide studies. The level of left to right shunt was accurately assessed in all patients, and its absence observed postoperatively. Associated anomalies, such as a persistent left superior vena cava, coarctation of the aorta and partial anomalous venous return, were identified in all cases.(ABSTRACT TRUNCATED AT 250 WORDS)

Repair of Atrial Septal Defect with Partial Anomalous Pulmonary Venous Return from the Left Upper Lobe

A patient with partial anomalous venous return from the left upper lobe in the presence of a large atrial septal defect is presented. The diagnosis was confirmed by selective angiographic studies. Two technical points are emphasized: first, that the exposure through a midsternal incision is excellent, and second, that the anomalous vein must be detached from its connection to the innominate or subclavian artery as high into the left chest as it is technically possible to reach. Postoperative cardiac catheterization and angiograms confirm the completeness of the repair.

Transaortic repair of double-outlet right ventricle with situs inversus, 1-loop, 1-malposition (I,L,L), subaortic ventricular septal defect, and associated anomalies

Successful total correction of a rare form of double-outlet right ventricle (I.L.L.) is reported wherein transaortic construction of the ventricular baffle was used. Surgical approach to the defect was unsatisfactory through a right ventriculotomy. Excellent exposure was achieved through the aortic valve. Correction of partial anomalous venous return from the left lung, pulmonary debanding, and repair of the pulmonary artery were also done. Double superior venae cavae made special venous cannulation necessary. Cardiac catheterization at one year documented successful repair. This case illustrates the usefulness of the transaortic approach to a ventricular septal defect when repair through a ventriculotomy is difficult or impossible.

"Bridging Bronchus"

This report describes the clinical features and pathologic anatomy seen in an infant with anomalous bronchial branching and other congenital anomalies. The patient died of respiratory insufficiency and infection, possibly fostered by the malformed airway and a coexistent partial anomalous venous return. At autopsy, bronchial drainage of the right middle and lower lobes was provided by a large bronchial branch that originated in the left main-stem bronchus and bridged the mediastinum before entering the contralateral lung. Since crossed anatomic airway relationships after formation of primary branches are imcompatible with current concepts of embryologic lung development, a brief critical review of the pertinent literature is undertaken.

EARLY MANIFESTATIONS OF THE CARDIOVASCULAR DISORDERS IN MARFAN SYNDROME

We have studied four children varying in age from 5 months to 26 months with typical features of Marfan syndrome. Aside from the routine clinical and laboratory procedures, phonocardiograms, cardiac catheterizations, angiocardiograms and retrograde aortograms were performed. All patients had demonstrable aneurysms of the aortic sinuses of Valsalva and dilatation of the ascending aorta and of the main pulmonary artery. One case had a partial anomalous venous return to the innominate vein. The phonocardiograms demonstrated accentuated pulmonic closures, mid or late systolic murmurs and in one case an apical pansystolic murmur. The anomalies found at necropsy in one infant at the age of 12 days and other congenital heart defects in three older patients are reported. The general aspects of the disease are discussed and a review of the associated cardiac lesions in previously reported cases is presented. Attention is called to the minimal clinical findings of serious underlying cardiac lesions at a very early age, which can only be demonstrated by angiocardiography and retrograde aortography. To our knowledge these are the youngest patients in whom these cardiovascular lesions were demonstrated during life.

Contributions of open cardiotomy to the correction of congenital and acquired cardiac disease.

Partial and Total Anomalous Pulmonary Venous ReturnAs mentioned above, it has been uncommon for partial anomalous venous return to exist without at least a patent foramen ovale, and more often it is associated with a secundum type of atrial septal defect.Obviously, all cases of total anomalous venous return require the coexistence of an atrial shunt. The operative management of total anomalous insertion of the pulmonary veins is therefore one uniformly complicated by the presence of an atrial septal defect. Since ideally the defect can only be corrected by direct-vision intracardiac reparative surgery, the management of anomalous venous return . . .