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Query: UMLS:C0018799 (heart disease)
 34,133 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Between January, 1967, and October, 1977, we performed intracardiac repair in 24 patients with unroofed coronary sinus. Eight patients had the so-called pure form of the syndrome, with the concomitant findings of total absence of the partition between the coronary sinus and left atrium, connection of the left superior vena cava (LSVC) to the upper left corner of the left atrium, and a coronary sinus type of atrial septal defect. In 2 additional patients this combination was repaired, along with repair of the common atrium in 1 and tetralogy of Fallot in the other. In 4 patients with either a partial or complete atrioventricular canal defect, only the distal or downstream portion of the sinus was unroofed so that the coronary sinus ostium was considerably to the left of its usual location. In 6 patients, 5 of whom had situs ambiguous, the unroofed coronary sinus was associated with complex congenital heart disease, and in 5 there was a L(contralateral) SVC. Three of these 6 patients died. The basic repair consists of roofing the coronary sinus from within the left atrium, so that the LSVC drains through the newly created coronary sinus and its ostium into the right atrium. In the absence of a LSVC, the basic repair is simply closure of the coronary sinus atrial septal defect. This leaves the coronary veins draining into the left atrium.
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PMID:Surgical experience with unroofed coronary sinus. 45 16

Two children (aged 12 and 14 yr) with a history of complex congenital heart disease presented with symptoms and signs suggestive of pulmonary embolism. Initial ventilation-perfusion lung scans showed normal, approximately equal ventilation to both lungs. Global reduction of perfusion to the right lung was observed in one and was observed in the left lung in the other patient. It was not possible to exclude a large, central embolus in either case. Due to complex cardiac anatomy, however, which included bilateral cavopulmonary anastomoses in the first patient and SVC-right pulmonary artery and right atrial-pulmonary artery anastomoses in the second, repeat scans were performed within a short period in each case with different injection sites, including the contralateral arm and a leg injection, respectively. In both patients, these follow-up scans showed a different perfusion agent distribution to each lung when compared to the initial scans. These results demonstrated that there was no evidence of pulmonary embolism in either case.
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PMID:Pseudo pulmonary embolism in complex congenital heart disease. 870 73

The purpose of this study was to investigate the efficacy and safety of atrial cardioversion using an endocardial single lead system presently used for ventricular defibrillation. The study population consisted of 26 recipients of an ICD in combination with a conventional endocardial single lead system with the proximal spring electrode as anode in the SVC and the distal as cathode in the apex of the RV. Atrial tachyarrhythmias were induced by right atrial burst pacing. If the arrhythmia sustained > 1 minute, biphasic shocks synchronized with the R wave were delivered using the implanted device, beginning with an energy of 4 J. If 4 J failed to terminate the arrhythmia, energy was increased stepwise, if the first shock was successful, a step-down testing was performed after reinduction of atrial tachyarrhythmias. The mean atrial defibrillation threshold was 2.3 +/- 1.2 J (range, 0.5-5 J). A total of 154 shocks were delivered and no adverse effects were observed. The mean defibrillation threshold for atrial flutter was somewhat lower than that for AF (1.8 +/- 1 J vs 2.7 +/- 1.4 J, P = 0.08). There was no correlation between the atrial defibrillation threshold and a history of previously occurring atrial tachyarrhythmias, the kind of the underlying heart disease, a prescription of antiarrhythmic drugs, the dimension of the LA, the LVEF, or the ventricular DFT. Internal atrial cardioversion of short duration atrial tachyarrhythmias using a transvenous single lead system designed for ventricular defibrillation is feasible and safe at low energies, and may have important clinical applications.
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PMID:Low energy transvenous cardioversion of short duration atrial tachyarrhythmias in humans using a single lead system. 912 73

The incidence of persistent left superior vena cava (PLSVC) is approximately 0.5% in the general population; however, the coexistent absence of the right SVC has a reported incidence in tertiary centers of 0.1%. The vast majority of reports are limited to pediatric cardiology. Likewise, sinus of Valsalva aneurysm is a rare congenital anomaly, with a reported incidence of 0.1-3.5% of all congenital heart defects. We present a 71-year-old patient undergoing preoperative evaluation for incidental finding of aortic root aneurysm, and found to have all three in coexistence. Suggestive findings were demonstrated on cardiac catheterization and definitive diagnosis was made by magnetic resonance imaging. The use of MRI for the diagnosis of asymptomatic adult congenital heart disease will be reviewed.
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PMID:Utility of magnetic resonance imaging in cardiac venous anatomic variants. 1456 86

Diagnostic testing in patients with congenital heart disease is usually performed supine and at rest, conditions not representative of their typical hemodynamics. Upright exercise measurements of blood flow may prove valuable in the assessment of these patients, but data in normal subjects are first required. With the use of a 0.5-T open magnet, a magnetic resonance-compatible exercise cycle, and cine phase-contrast techniques, time-dependent blood flow velocities were measured in the right (RPA), left (LPA), and main (MPA) pulmonary arteries and superior (SVC) and inferior (IVC) vena cavae of 10 healthy 10- to 14-yr-old subjects. Measurements were made at seated rest and during upright cycling exercise (150% resting heart rate). Mean blood flow (l/min) and reverse flow index were computed from the velocity data. With exercise, RPA and LPA mean flow increased 2.0 +/- 0.5 to 3.7 +/- 0.7 (P < 0.05) and 1.6 +/- 0.4 to 2.9 +/- 0.8 (P < 0.05), respectively. Pulmonary reverse flow index (rest vs. exercise) decreased with exercise as follows: MPA: 0.014 +/- 0.012 vs. 0.006 +/- 0.006 [P = not significant (NS)], RPA: 0.005 +/- 0.004 vs. 0.000 +/- 0.000 (P < 0.05), and LPA: 0.041 +/- 0.019 vs. 0.014 +/- 0.016 (P < 0.05). SVC and IVC flow increased from 1.5 +/- 0.2 to 1.9 +/- 0.6 (P = NS) and 1.6 +/- 0.4 to 4.9 +/- 1.3 (P < 0.05), respectively. A 56/44% RPA/LPA flow distribution at both rest and during exercise suggests blood flow distribution is dominated by distal pulmonary resistance. Reverse flow in the MPA appears to originate solely from the LPA while the RPA is in relative isolation. During seated rest, the SVC-to-IVC venous return ratio is 50/50%. With light/moderate cycling exercise, IVC flow increases by threefold, whereas SVC remains essentially constant.
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PMID:Blood flow conditions in the proximal pulmonary arteries and vena cavae: healthy children during upright cycling exercise. 1503 Nov 21

To avoid fatal complications of central venous catheterization such as cardiac tamponade, the tip of the central venous catheter (CVC) should be placed outside of the cardiac chamber. To suggest a guideline for a proper depth of CVC in infants, we measured the distance from the skin puncture site to the junction between superior vena cava and right atrium (SVC-RA junction) by using transesophageal echocardiography (TEE). Fifty infants less than 5 kg undergoing surgery for congenital heart disease were enrolled in this prospective study. After the induction of general anesthesia, CVC was inserted via the right subclavian vein. After the tip of the CVC was placed at the SVC-RA junction using TEE guidance, the length of the CVC inserted beneath the skin was measured. The measured distance had a high correlation with the patient's height, weight, and age (r = 0.88, 0.76, and 0.64, respectively). In infants smaller than 5 kg, the following guideline can avoid intraatrial placement of the CVC: a depth between 40 and 45 mm for infants 2.0-3.0 kg in weight, 45-50 mm for those 3.0-3.9 kg, and 50-55 mm for those more than 4.0 kg.
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PMID:The optimal depth of central venous catheter for infants less than 5 kg. 1624 84

Persistent left-sided superior caval veins (SVC) are present in 0.4% of the population. In the majority of cases, the persistent left SVC drains into the right atrium via the coronary sinus, but direct connection to the left atrium is also recognized. Previous reports have described re-opening of persistent left SVCs in patients with congenital heart disease following bidirectional cavopulmonary connection or Fontan-type procedures, suggesting that the lumen of the left SVC obliterates during embryological development, rather than disappears. The case described in this report is, to our knowledge, the first description of obliteration of the left SVC in post-natal life, associated with spontaneous closure of a ventricular septal defect. Our observation lends further support to the hypothesis that venous structures obliterate but do not completely disappear in foetal life.
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PMID:Obliteration of left superior caval vein draining to the left atrium during spontaneous closure of ventricular septal defect. 1875 61

Persistence of a left-sided superior vena cava (PLSVC) with absent right superior vena cava (isolated PLSVC) is a very rare venous malformation and commonly associated with congenital heart disease or alterations of the cardiac situs. We describe an unusual case of a young boy presenting with persistent atrial tachycardia and congestive heart failure. He was detected to have unexplained grossly dilated right atrium, right ventricle with systolic dysfunction and a giant coronary sinus (CS). The dilated CS closely mimicked a pseudo cor-triatriatum on echocardiography. Contrast echocardiography from both arms revealed opacification of the CS before the right atrium. Bilateral upper limb venography confirmed the presence of absent right SVC and isolated persistent left SVC draining into the giant coronary sinus.
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PMID:Isolated persistent left-sided superior vena cava, giant coronary sinus, atrial tachycardia and heart failure in a child. 2420 85

Staged palliation to achieve a total cavopulmonary connection is a common treatment strategy in patients with single ventricle congenital heart disease. Patients with bilateral superior caval veins (bilateral SVC) often require the creation of bilateral superior cavopulmonary connections as part of the staged palliation, and these patients are at increased risk of morbidity. We describe a novel technique used in two patients with bilateral SVC and very small (1-2 mm) bridging vein that encouraged bridging vein growth and facilitated creation of a unilateral superior cavopulmonary connection.
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PMID:Superior Vena Cava Banding to Facilitate Unilateral Bidirectional Glenn Operation in Patients With Single Ventricle Heart Disease and Bilateral Superior Caval Veins. 2792 43

Persistent left-sided superior vena cava (PLSVC) is the commonest systemic venous anomaly in the thorax with a reported prevalence of up to 0.5% in otherwise normal population and up to 10% in patients with congenital heart disease (CHD). In the absence of associated CHD, it is usually asymptomatic, discovered incidentally. It may complicate catheter or pacemaker lead placement. PLSVC typically drains into the right atrium through the coronary sinus. In children with CHD, the presence of a PLSVC may affect the choice of certain surgical procedures. PLSVC is significantly more common in association with situs ambiguous than with situs solitus or inversus, up to 60-70%. In patients with situs ambiguous, the drainage of LSVC is variable, more commonly directly into the atria rather than through the coronary sinus (CS). Rarely, there is a PLSVC draining into the CS with absent right SVC. PLSVC draining into the right atrium via the CS will not usually cause blood shunting between the right and the left sides. However, shunting occurs when PLSVC is associated with unroofed CS, or when it directly drains into the left atrium. With an increased use of CT and MRI for chest and cardiac imaging, PLSVC is being more encountered by radiologists than before. In this article, we will discuss the embryology of PLSVC, its anatomic course and drainage pathways, as well as its clinical relevance and relation to congenital heart disease and viscero-atrial situs.
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PMID:Left superior vena cava: cross-sectional imaging overview. 3182 96


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