Cross-sectional and contrast echocardiography in the diagnosis of interatrial communications through the coronary sinus

An interatrial communication through the mouth of the coronary sinus is a rare cardiac anomaly. It is usually associated with a left superior caval vein draining into the coronary sinus and is frequently part of a complex congenital malformation of the heart. Failure to recognize this defect before surgical repair of the primary cardiac lesion may lead to persistent interatrial shunting, which may in turn cause postoperative cyanosis. This report describes two cases of interatrial communication through the coronary sinus diagnosed prospectively by echocardiography. It demonstrates the important role of contrast echocardiography in the evaluation of patients suspected of having this malformation.     

Reopening of a persistent left superior vena cava in the early postoperative period following bidirectional cavopulmonary anastomosis--treatment by coil embolization]

Diagnosis of systemic venous drainage is mandatory for patients with congenital heart disease planned for cavopulmonary anastomosis or any Fontan-type palliation. Preexisting venous channels as the persistent left superior vena cava are common in cardiac anomalies and may lead to postoperative deterioration and cyanosis. We describe a 6 month old boy with a complex imbalanced atrioventricular septal defect who developed cyanosis in the very early postoperative period, following bidirectional cavopulmonary anastomosis. It was caused by ineffective lung perfusion due to a reopened persistent left superior vena cava with drainage to the coronary sinus.He underwent coil embolization of the persistent left superior vena cava with retrievable coils and cyanosis improved. Coil embolization is an effective alternative to secondary surgery, especially for hemodynamically compromised patients in the postoperative period.     

Catheter closure of coronary sinus atrial septal defect using Amplatzer Septal Occluder

Coronary sinus defect is a rare type of atrial septal defect. We report two patients who had a coronary sinus atrial septal defect without persistent left superior caval vein, where the orifice of the coronary sinus was closed using the Amplatzer Septal Occluder. The procedure was successful, without any complications including conduction disturbance.     

Missed unroofed coronary sinus

Coronary sinus atrial septal defect (ASD) is a rare congenital cardiac anomaly, which might be difficult to diagnose. In this report, we describe a patient with small secundum ASDs and an associated large coronary sinus ASD, which had been missed at initial evaluation. The diagnosis of coronary sinus ASD was established by using transesophageal echocardiography after percutaneous closure of a small secundum ASD at another center. Patient underwent corrective surgery.     

Is routine preoperative cardiac catheterization necessary before repair of secundum and sinus venosus atrial septal defects?

Between January 1976 and July 1983, 217 patients with atrial septal defect underwent surgical repair at Children's Hospital. Thirty with a primum atrial septal defect and 26 who underwent cardiac catheterization elsewhere before being seen were excluded from analysis. Of the 161 remaining patients, 52 (31%) underwent preoperative cardiac catheterization, 38 because the physical examination was considered atypical for a secundum atrial septal defect and 14 because of a preexisting routine indication. One hundred nine (69%) underwent surgery without catheterization, with the attending cardiologist relying on clinical examination alone in 5, additional technetium radionuclide angiocardiography in 5, M-mode echocardiography in 13 and two-dimensional echocardiography in 43; both M-mode echocardiography and radionuclide angiography were performed in 24 and two-dimensional echocardiography and radionuclide angiography in 19. Since 1976, there has been a trend toward a reduction in the use of catheterization and use of one rather than two noninvasive or semiinvasive techniques for the detection of atrial defects. Of the 52 patients who underwent catheterization, the correct anatomic diagnosis was made before catheterization in 47 (90%). Two patients with a sinus venosus defect and one each with a sinus venosus defect plus partial anomalous pulmonary venous connection, partial anomalous pulmonary venous connection without an atrial septal defect and a sinoseptal defect were missed. Of 109 patients without catheterization, a correct morphologic diagnosis was made before surgery in 92 (84%). Nine patients with a sinus venosus defect, three with sinus venous defect and partial anomolous pulmonary venous connection, four with partial anomalous pulmonary venous return without an atrial septal defect and one with a secundum defect were incorrectly diagnosed.(ABSTRACT TRUNCATED AT 250 WORDS)     

Iatrogenic cyanosis and clubbing: 25 years of chronic hypoxia after the repair of an atrial septal defect

A case of sinus venosus atrial septal defect repair of the inferior vena caval type with an unintentional diversion of the inferior vena caval blood to the left atrium is reported. Long-standing, anatomical, right-to-left shunting with cyanosis and hypoxia are associated with systemic and cerebrovascular complications. Cardiac risks depend on the presence or absence of pulmonary hypertension, the associated hematological abnormalities and the degree of anatomical, right-to-left shunting. Cardiac magnetic resonance imaging clarified the etiology of the unexplained cyanosis and delineated the surgical anatomy.     

Absent right and persistent left superior vena cava

A patient is described presenting with atrial fibrillation. A dilated coronary sinus was found due to the presence of a persistent left superior caval vein. Absence of the right superior caval vein was suspected with contrast injection through an i.v. line in the right arm, and was confirmed with phlebography. No associated cardiac anomalies were found. Persistent left superior vena cava is a common anomaly, although simultaneous complete absence of the right superior vena cava is rare. The incidence, embryology, diagnosis and importance of this anomaly is discussed.     

Platypnea-orthodeoxia syndrome combined with multiple congenital heart anomalies

A 71-year-old woman was admitted for paralysis on the left side of her body. She developed dyspnea and hypoxemia after admission. Although pulmonary embolism was suspected, hypoxemia and dyspnea occurred repeatedly in spite of anticoagulation therapy. Transesophageal echocardiography revealed a patent foramen ovale (PFO), an atrial septal aneurysm (ASA), and a right-to-left shunt that appeared in an upright position. She was diagnosed with platypnea-orthodeoxia syndrome. Moreover, cardiac catheterization showed congenital anomalies, such as unroofed coronary sinus, partial anomalous pulmonary venous return and persistent left superior vena cava. Simple surgical closure of the ASA and PFO improved all of her symptoms.     

Atrial septal defect with cyanosis without pulmonary hypertension or anomalous systemic venous drainage to the left atrium

A case of a 29-year-old patient with cyanosis due to a right-to-left shunt is reported. This patient presented an atrial septal defect within the oval fossa and an anomalous left superior caval vein. The direction of this shunt is explained by the anatomical relationship between the oval fossa and the dilated coronary sinus, draining venous blood into the left atrium across the atrial septal defect. Cyanosis disappeared after surgical correction of the defect.     

Diagnosis and Surgical Treatment of Congenital Cor Triatrium

Objectives To evalu-ate the outcome of diagnosis and surgical treatment for cor triatrium (CTA) in 6 patients seen between 1994 and 2002. Methods 6 patients ranging in age from 5 months to 25 years were observed. All of them had other cardiovascular defects, and presented with dyspnea, palpitations (and low weight growing only in the infants) . Preopreative two dimensional echocardiogra-phy had demonstrated an abnormal septum in the left atrium and other coexistent anomalies. In 3 of them the membrane was obstructed between the left atrial accessory chamber and the left atrium. The communication were atrial septal defect (ASD) indirectly; The clinical findings were due to the pulmonary hypervas-cularity. Only one case had a fenestration in the septum with a small patent foramen ovale (FO) directly , and the clinical findings were due to the obstruction to flow through the membrane in the left atrium, producing venocapilar pulmonary hypertension. Two of them had ASD and fenestration on the septum. The ot     

Association of Left Ventricular Diverticula and Sinus Venosus Atrial Septal Defect

Left ventricular diverticula are congenital anomalies and are not as rare as previously thought. In adults, cardiac diverticula are generally accidental findings during imaging modalities, but concomitant disorders might frequently coexist. The pathophysiology, management, prognosis, and natural history of cardiac diverticula remain poorly understood and controversial. Definite diagnosis is often challenging due to similarities in appearance to other more common anomalies such as aneurysms, pseudoaneurysms, endocarditis, cysts, and hypertrophied trabeculations. We herein report a rare case of an adolescent presenting with sinus venosus atrial septal defect, partial anomalous venous connection, and left ventricular diverticula.     

佛氏窦瘤破裂的外科治疗

佛氏窦瘤破裂(RASV)是一种较少见的心脏病,自1982年5月至1991年6月,我院共手术治疗18例,效果满意。     

Right atrial myxoma with atrial septal defect: A case report and review of the literature

We report the case of a 63-year-old woman presenting with progressive dyspnea of insidious onset culminating in severe central cyanosis. Conventional studies including M-mode echocardiography did not point to the diagnosis. At cardiac catheterization a large right atrial myxoma producing partial dynamic tricuspid obstruction was discovered along with an atrial septal defect with a right to left shunt. After successful surgical excision of the tumor and repair of the atrial septal defect, the patient has been totally relieved of her presenting symptoms.     

Deciphering the Mysteries of Crisscross Heart by Transthoracic Echocardiography

Background: Accurate diagnosis of crisscross heart and its associated anomalies is important but problematic for cardiologists. This study aimed at identifying unique transthoracic echocardiographic features and common associated lesions of this complex condition. Method: Clinical and echocardiographic features of 10 patients with crisscross anatomy were studied. Echocardiographic findings were verified by cardiac magnetic resonance imaging or surgical inspection. Results: Crisscross anatomy (10 patients, age at diagnosis ranged from 1 month to 25 years, five female) was identified in 0.076% of patients with congenital heart diseases from 1985 to 2006. All patients had cyanosis and 80% of them were underweight. Superior–inferior ventricles (SIV) and crossed ventricular inflow streams were seen in 90% and 100% of patients, respectively. All patients had abnormal ventriculo–arterial (VA) connections: five with transposition of great artery (L‐type: n = 2; D‐type: n = 3) and five with double outlet right ventricle. Commonly associated anomalies included ventricular septal defects (100%), right ventricular outflow tract obstruction (60%), atrioventricular valves straddling or overriding (50%), atrial septal defect (40%), and right ventricular hypoplasia (30%). Seven patients received cardiac surgery for the relief of cyanosis. Conclusions: SIV and crossed inflow streams are important diagnostic features for crisscross heart by transthoracic echocardiogram. The hemodynamic consequences of abnormal VA connections and associated defects impact surgical management. (Echocardiography 2011;28:104‐108)     

Atrial septal defect with intermittent hypoxia due to venous return through a persistent left superior vena cava return draining into a contiguous coronary sinus. Case report.

A case is reported of a 38-month-old female patient with an ostium secundum-type atrial septal defect, 25 mm in diameter, with intermittent hypoxemia and hypoplasia of the right ventricular cavity and tricuspid valve. The right-to-left shunt through the atrial septal defect, responsible for these alterations, is explained by its proximity to the coronary sinus, and exacerbated by a persistent left superior vena cava. The cyanosis disappeared and normal hemodynamic status was achieved after closure of the atrial septal defect.     

Atrial septal defect with intermittent hypoxia due to venous return through a persistent left superior vena cava return draining into a contiguous coronary sinus. Case report.

A case is reported of a 38-month-old female patient with an ostium secundum-type atrial septal defect, 25 mm in diameter, with intermittent hypoxemia and hypoplasia of the right ventricular cavity and tricuspid valve The right-to-left shunt through the atrial septal defect, responsible for these alterations, is explained by its proximity to the coronary sinus, and exacerbated by a persistent left superior vena cava. The cyanosis disappeared and normal hemodynamic status was achieved after closure of the atrial septal defect.     

Right heart failure due to an unroofed coronary sinus in an adult

An unroofed coronary sinus is a rare congenital malformation with a strong association to a persistent left vena cava superior. In most cases it occurs together with complex cardiac anomalies such as left isomerism. We report on a 72-year-old man with clinical signs of right heart failure caused by an isolated unroofed coronary sinus: The diagnosis was made by means of transesophageal echocardiography with contrast injection.     

A rare association recognized before cardiac surgery: persistent left superior vena cava and secundum atrial septal defect

One of the most common congenital anomalies of systemic veins is persistent left superior vena cava. Association of persistent left superior vena cava with other congenital cardiac diseases is common and frequently encountered during diagnostic studies. Contrast echocardiography has an important role in the diagnosis. Owing to the fact that cardiopulmonary by-pass procedure may be problematic in patients with persistent left superior vena cava, this anomaly should be detected before cardiac surgery and required measures should be taken. Our case is an association of persistent left superior vena cava detected in a patient to be operated for secundum atrial septal defect. We report the case owing to its low frequency and to emphasize the importance of detection before cardiac surgery.     

Stenting for superior caval vein stenosis after surgical repair of sinus venosus atrial septal defect

A young woman had symptoms from stenosis of the superior caval vein 8 years after surgical repair of sinus venosus atrial septal defect with partial anomalous pulmonary venous drainage. She was successfully treated by balloon dilatation and stenting.     

Severe Right Ventricular Outflow Obstruction by Right Sinus of Valsalva Aneurysm

Aneurysms of the sinus of Valsalva are rarely diagnosed cardiac anomalies, occurring in 0.14%–0.96% of patients who have undergone open heart surgical procedures. The most common congenital anomalies accompanying sinus of Valsalva aneurysm (SVA) are ventricular septal defect, bicuspid aortic valve, atrial septal defect, and coarctation of aorta. We report a patient with an unruptured right SVA presenting with severe right ventricular outflow tract (RVOT) obstruction, and coexisting patent foramen ovale (PFO) with a right to left shunt. It could be assumed that the increase in right atrial pressure due to RVOT obstruction had led to a right to left shunt across the patent foramen ovale. (Echocardiography 2010;27:341‐343)