Rupture of a giant saccular aneurysm of coronary arteriovenous fistulas

A 58-year-old Japanese woman was admitted to our hospital because of chest pain. A continuous murmur was detected at the left parasternal area. Electrocardiogram showed ST elevation in leads V2, V3 and V4. Chest computed tomography and echocardiography demonstrated pericardial effusion and a large mass which was adjacent to the pulmonary artery. An abnormal blood flow was detected in the mass by Doppler echocardiography. Coronary angiography confirmed that the mass was a giant aneurysm of coronary arteriovenous fistula arising from both the left and right coronary arteries. This patient had no symptoms until rupture of the fistula. Rupture of a coronary arteriovenous fistula is very rare but can be a cause of chest pain and pericardial effusion.     

Rare case of pulmonary arteriovenous fistula simulating residual defect after transcatheter closure of patent foramen ovale for recurrent paradoxical embolism.

We report on a patient suffering from recurrent cerebrovascular events despite previous transcatheter closure of persistent foramen ovale (PFO) with a Helex occluder. There was evidence of persistent left-to-right atrial shunt shown by transesophageal contrast echocardiography and the patient was admitted to our institution for interventional closure of the supposed residual defect. However, the PFO was completely closed by the device and left pulmonary artery injections showed a pulmonary arteriovenous fistula in the left lower lobe. This rare malformation may well explain the recurrent paradoxical embolism. Transcatheter fistula closure with coils was performed successfully. This case underlines that the existence of an isolated pulmonary arteriovenous fistula as a right-to-left shunt in patients with cryptogenic stroke should not be overlooked, even if a PFO is present and pulmonary arteriovenous fistula is not suggested by the initial physical findings or chest X-ray.     

Platypnea - orthodeoxia syndrome with atrial septal defect.

A 75-year old man was referred to hospital for symptomatic hypoxemia. He did not complain of dyspnea while supine, but while sitting or standing, he experienced dyspnea with severe hypoxemia. He did not have any pulmonary diseases that could cause dyspnea. Transesophageal echocardiography revealed an atrial septal aneurysm with a small atrial septal defect (ASD) and a mild left-to-right shunt through the ASD when the patient was supine. However, when he became upright, a severe right-to-left shunt occurred and the arterial oxygen saturation decreased from 96% to 80% with dyspnea. Cardiac catheterization revealed normal pulmonary artery pressure. He was therefore diagnosed as having platypnea - orthodeoxia syndrome. Magnetic resonance imaging of the chest showed a deformity of the atrium associated with elongation of the ascending aorta. The ASD was closed surgically and the dyspnea and hypoxemia that occurred while he was upright completely resolved.     

Pulmonary arteriovenous fistula in a patient with renal cell carcinoma.

Arteriovenous (AV) fistulas are common in renal cell carcinoma. These are usually small and asymptomatic. Case reports have previously described symptomatic fistulas in primary renal cell tumour or, less commonly, bone metastases. The current study describes a patient with lung metastases from renal cell carcinoma who developed a symptomatic pulmonary AV fistula. The patient presented with debilitating progressive shortness of breath, which resolved after coiling of the pulmonary AV fistula. Supporting radiographs include a unique ventilation-perfusion finding of perfusion tracer in the kidney that is diagnostic of a pulmonary shunt. This is the first report of a pulmonary fistula from renal cell carcinoma. This rare complication of renal cell carcinoma reflects its diverse clinical presentations and unique tumour biology.     

Successful thoracoscopic resection of pulmonary arterio-venous fistula]

A 65-year-old female was admitted for further examination of an abnormal shadow on a chest roentgenogram. Chest computed tomography revealed a lobular nodule with blood vessels, which was diagnosed as pulmonary arterio-venous fistula (PAVF). Thoracoscopic resection was performed under general anesthesia. The fistula was situated directly below the pleura of the left lower lobe. Partial resection of the left lung including the PAVF was performed with an autosuture device dedicated to endoscopic surgery (Endo GIA II). The patient was discharged from our hospital 3 days after surgery without major complications. Mild hypoxemia was corrected after the surgery. Thoracoscopic resection is considered to be safe and useful for a solitary PAVF existing a peripheral field of the lung.     

Two-dimensional echocardiographic spectrum of univentricular atrioventricular connection

The spectrum of anomalies in hearts having a univentricular atrioventricular (AV) connection was examined by two-dimensional echocardiography in 183 patients and the anatomic findings were compared with angiography. The mode of AV connection was found to be of three types: double inlet via two AV valves; single inlet via one AV valve with absence of the other (left or right AV valve atresia); and common inlet via a common AV valve. Identification of an accessory chamber by two-dimensional echocardiography was possible with 90% sensitivity, but it was limited compared with angiography in patients with severely hypoplastic anterior chambers and pulmonary valve atresia. All patients with subaortic outlet foramen obstruction were detected. Great artery position and the presence of obstruction to pulmonary flow were correctly predicted in all but one patient. Two-dimensional echocardiography was superior to angiography for the detection of AV valve abnormalities which were present in 27% and included abnormal chordae, hypoplasia or dysplasia of either valve. Two-dimensional echocardiography should play an essential role in the complete preoperative assessment of patients with univentricular AV connection.     

Transthoracic Echocardiography for Diagnosis of Right Pulmonary Artery to Left Atrial Fistula

Right pulmonary artery to left atrial fistula (RPA‐LAF) is a rare cardiovascular anomaly. There were no systematic and detail represent by echocardiography. We chose the patients who diagnosed with RPA‐LAF at Fuwai Hospital from 2000 to 2010. All patients underwent clinical exanimation, chest roentgenogram, laboratory testing, electrocardiography, transthoracic echocardiography (TTE), contrast echocardiography, and cardiac catheterization. In this article, we summarize the characteristics of the TTE for diagnosing the rare cardiovascular anomaly of RPA‐LAF. We undertook a detailed review of their TTE and contrast echocardiography findings to determine the characteristic findings of this condition.     

Hereditary telangiectasia and multiple pulmonary arteriovenous fistulas. Clinical deterioration during pregnancy

We describe the effect of pregnancy on a woman with multiple pulmonary arteriovenous fistula. Pregnancy was terminated at 35 weeks' gestation because of severe hypoxemia. During the early postpartum period, the intrapulmonary shunt fraction enlarged, and hypoxemia worsened, necessitating emergency resection of the A-V fistula. Pregnancy may increase the intrapulmonary shunt fraction in patients with multiple pulmonary arteriovenous fistula through its effect on plasma volume and produce life-threatening hypoxemia near term or in the early postpartum period.     

Two-dimensional contrast echocardiography in pulmonary arteriovenous fistula

Definitive clinical diagnosis of congenital pulmonary arteriovenous fistula is extremely difficult. In order to evaluate the diagnostic value of echocardiography, 2 cases with suspected pulmonary arteriovenous fistula were studied. In the first case, there was a solitary pulmonary arteriovenous fistula, while in the second multiple minute pulmonary arteriovenous fistulas were illustrated. The solitary lesion was demonstrated by two-dimensional and peripheral vein contrast echocardiography. However, in the second case direct visualization of the lesion was not possible but peripheral vein contrast echocardiography showed abnormal filling of the left atrium with echo contrast material.     

Potential Faces of Patent Foramen Ovale (PFO PFO)

Background: Patent foramen ovale (PFO) is diagnosed on echocardiography by saline contrast study with or without color Doppler evidence of shunting. PFO is benign except when it causes embolic events. Methods and Results: In this report, we describe unique additional manifestations related to the diagnosis and presentation of PFO. These include demonstration of PFO during the release phase of “sigh” on the ventilator in the operating room, use of a separate venipuncture to allow preparation of blood‐saline‐air mixture after multiple failed saline bubble injections, resting and stress hypoxemia related to left to right shunting across a PFO in the absence of pulmonary hypertension, presentation of quadriperesis secondary to an embolic event from a PFO and development of a thrombus on the left atrial aspect of PFO in a patient with atrial fibrillation, and on the right atrial aspect of PFO in a patient who had undergone repair of a flail mitral valve. Finally, in one patient with end‐stage renal disease, aortic valve endocarditis and periaortic abscess, PFO acted as a vent valve relieving right atrial pressure following development of aortoatrial fistula. Conclusion: PFO diagnosis can be elusive if appropriate techniques are not used during saline contrast administration. PFO can present as hypoxemia in the absence of pulmonary hypertension, can be a rare cause of quadriperesis, and can be associated with thrombus formation on either side of interatrial septum. Finally, PFO presence can be lifesaving in those with sudden increase in right atrial pressure such as with aortoatrial fistula. (Echocardiography 2010;27:897‐907)     

Aortopulmonary Artery Fistula: Ruptured Aneurysm of the Distal Aortic Arch into the Pulmonary Artery

Aortopulmonary artery fistula is uncommon, but the clinical outcome is often lethal. A 76‐year‐old man with a history of acute thoracic aortic dissection 6 years previously was admitted with dyspnea. A chest x‐ray showed pleural effusion and pulmonary congestion. Transthoracic echocardiography revealed preserved systolic function, but continuous and abnormal flow from the distal aortic arch into the pulmonary artery (PA). Transesophageal echocardiography (TEE) in the Doppler color‐flow mode demonstrated a left‐to‐right shunt between a large distal aortic arch aneurysm and the left PA via an aortopulmonary fistula and a pressure gradient across the shunt of 56 mmHg. Contrast‐enhanced computed tomography showed that the aneurysm compressed the PA. Aortography also revealed a large distal aortic arch aneurysm and almost simultaneous contrast enhancement of the aorta and the PA. Right‐heart catheterization showed a significant increase in oxygen saturation between the right ventricle and the PA. A left‐to‐right shunt due to a distal aortic arch aneurysm rupturing into the left PA was diagnosed based on these findings. TEE was very helpful in confirming the presence and precise location of the fistula.     

Comparative value of transthoracic and transesophageal echocardiography in the assessment of congenital abnormalities of the atrioventricular junction

Information obtained from transthoracic and transesophageal echocardiography (two-dimensional echocardiography with spectral Doppler and color flow imaging) was compared in 17 patients with major congenital abnormalities of the atrioventricular (AV) junction (10 discordant AV connections, 1 criss-cross connection, 5 absent right connections and 1 absent left connection). The findings by either technique were correlated with findings at cardiac catheterization (12 patients) and at surgery (5 patients). In two of six patients with an absent AV connection as defined by transthoracic echocardiography, transesophageal imaging demonstrated an imperforate AV valve. In 11 of 11 patients with a discordant or criss-cross connection, assessment of AV valve and ventricular morphology (by defining the chordal attachments of both AV valves) was possible with transesophageal echocardiography (3 of 11 patients by transthoracic echocardiography); chordal straddling was detected in 1 patient and excluded in 3 others with an associated inlet ventricular septal defect. Anomalous pulmonary venous connection (one patient), atrial septal defect (three patients) and subpulmonary stenosis (five patients) were better assessed by transesophageal imaging, and atrial appendage morphology could be demonstrated in all. The transesophageal technique was less useful in demonstrating the anterior subaortic infundibulum or aortopulmonary shunt (two patients). Although systemic ventricular function could be assessed by either method with use of short-axis M-mode scans, transesophageal pulsed Doppler interrogation of AV valve and pulmonary venous flow patterns provided clues to diastolic dysfunction of the systemic ventricle.     

Pulmonary vascular manifestations of hereditary hemorrhagic telangiectasia (rendu-osler disease)

Hereditary hemorrhagic telangiectasia (HHT) or Rendu-Osler-Weber disease is a genetic disorder with autosomal dominance and variable penetrance, characterized by epistaxis, telangiectasia and visceral manifestations of the disease. The estimated minimal prevalence is 1/10,000 inhabitants. The diagnosis is established on clinical criteria, and may be further confirmed by the identification of causative mutations in either the ENG or the ACVRL1 gene coding for endoglin and ALK1, respectively. Pulmonary vascular manifestations of HHT include pulmonary arteriovenous malformations (PAVMs; especially in patients with ENG mutations) and less frequently pulmonary hypertension (especially in patients with ACVRL1 mutations). In 15-33% of patients with HHT, PAVMs consist of abnormal communications between pulmonary arteries and pulmonary veins, causing right-to-left shunting, and thus, frequently hypoxemia and dyspnea on exertion, although PAVMs may remain asymptomatic and frequently undiagnosed unless complications occur. PAVMs result in severe and frequent complications often at a young age, which may reveal the diagnosis, e.g. transient ischemic attack and cerebral stroke (10-19% of patients), systemic severe infections and abscesses (including cerebral abscess in 5-19% of patients), and rarely massive hemoptysis or hemothorax. Infections in HHT are related to the right-to-left shunting that bypasses the pulmonary capillaries and facilitates the passage of septic or aseptic emboli into the systemic and especially cerebral circulation, and potentially to minor defects in innate immunity. Treatment of PAVMs based on transcatheter coil vaso-occlusion of the feeding artery significantly decreases right-to-left shunting, hypoxemia and dyspnea on exertion, and reduces the risk of systemic complications. Long-term follow-up is warranted after transcatheter vaso-occlusion of PAVMs due to frequent recanalization of treated PAVMs and development or growth of untreated PAVMs. Patients with HHT should be informed of the risk of PAVM and potentially severe complications occurring in heretofore asymptomatic subjects. All adult patients with HHT should be proposed systematic screening for PAVM, by contrast echocardiography (preceded by anteroposterior chest radiograph) or computed tomography of the chest. Pulmonary hypertension is rare in HHT, and may be due either to systemic arteriovenous shunting in the liver increasing cardiac output or be clinically and histologically indistinguishable from idiopathic pulmonary arterial hypertension. Pulmonary hypertension is detected by systematic examination of right cardiac cavities and tricuspid regurgitation flow at echocardiography, and the diagnosis is established by right heart catheterization.     

Transcatheter brachial fistula creation for treatment of pulmonary arteriovenous malformations

Pulmonary arteriovenous malformations (PAVMs) are thought to form as a result of exclusion of hepatic venous blood from part of the pulmonary circulation. Surgical arteriovenous (AV) fistula creation has demonstrated therapeutic potential to reverse PAVM formation. We sought to demonstrate the feasibility and safety of transcatheter AV fistula creation for this indication. Fluoroscopically guided puncture from the basilic vein into the brachial artery using a Brockenbrough needle and sharpened 0.014” wire created a tract between these vessels. After balloon dilation of the tract, a covered stent was deployed, resulting in a functioning brachial AV fistula. The procedure was technically successful, with no clinical complications at 4 months follow‐up. Repeat diagnostic catheterization revealed marked improvement in systemic saturation and near‐resolution of PAVMs in the pilot patient. This report suggests that transcatheter brachial arteriovenous fistula formation is technically feasible, and may be effective in managing PAVMs in select single‐ventricle patients. © 2013 Wiley Periodicals, Inc.     

Body position and oxygenation: An intriguing relationship

Dyspnea and hypoxemia are among the most common symptoms and signs that need to be assessed in clinical practice.This case illustrates how simple steps in history taking and physical examination can be crucial for diagnosis.We present a patient with intermittent hypoxemia, initially attributed to a pulmonary infection. However, the hypoxemia persisted even after successful treatment of the infection. Computed tomography angiography of the chest and ventilation/perfusion lung scan excluded pulmonary embolism.We then observed that the hypoxemia and dyspnea were triggered by orthostatism. An echocardiogram with a bubble test showed a patent foramen ovale, with a right-to-left shunt, without pulmonary hypertension. After percutaneous closure of the foramen ovale, the symptoms completely resolved.This is a case of platypnea-orthodeoxia syndrome, which is usually associated with patent foramen ovale or atrial septal defect and is typically observed in the elderly. The features and causes of this curious syndrome are discussed.     

Intracardiac thrombosis in multiple chambers and descending aorta manifested as systemic and pulmonary thromboembolism

We report a case with multiple thrombosis that underwent sudden circulatory collapse due to acute pulmonary embolism and ischemic stroke. Further confirmed by transesophageal echocardiography, the thrombus in both atria was detected by transthoracic echocardiography. In addition, patent foramen ovale and another thrombus in the descending aorta were readily identified by transesophageal echocardiography. Aspirin was prescribed and thrombi disappeared 1 month later. Our case report suggests that patients undergoing sudden collapse with unexplained hypoxemia and new neurological deficit secondary to coexistence of pulmonary and systemic embolism should be promptly evaluated by echocardiography, especially transesophageal one, with high suspicion for paradoxical embolism.     

Atrioventricular canal defects

Opinion statement Atrioventricular (AV) canal defects should be diagnosed by means of echocardiography. Infants with complete AV canal defects should undergo intracardiac surgical repair before pulmonary vascular obstructive disease develops, preferably before 6 months of age. Repair of partial AV canal defects or primum atrial septal defects can be postponed until 18 to 36 months of age if the patient does not have pulmonary hypertension or growth failure. The one- and two-patch techniques can both provide excellent results with a low mortality rate in repair of complete AV canal defects. Repair of the cleft mitral valve at the time of initial surgery is advised to preserve long-term mitral valve function. Tetralogy of Fallot with AV canal defect can also be repaired but at an older age and with a greater incidence of reoperation. Unbalanced AV canal defects associated with a hypoplastic ventricle may necessitate initial pulmonary artery banding and a subsequent decision about the suitability of two-ventricle repair. If ventricular septation is unsuitable, a single-ventricle path is chosen. Bidirectional cavopulmonary anastomosis and, eventually, the Fontan procedure are used.     

Echocardiographic detection of coronary artery fistula into the pulmonary artery.

A rare case of left coronary artery--pulmonary artery fistula is reported. Transoesophageal echocardiography was capable of precisely demonstrating the origin, the course and the drainage site of the fistula. In contrast, transthoracic echocardiography could visualise a drainage flow in the pulmonary artery only. In conclusion, transoesophageal echocardiography may be helpful in the diagnosis or exclusion of the form of coronary artery fistula.     

Sinus node dysfunction unmasked during dobutamine stress echocardiography

Sinus bradycardia and associated isorhythmic AV dissociation may be seen during dobutamine stress echocardiography. This phenomenon is usually considered a benign peri-procedural event and is thought to be secondary to the Bezold-Jarisch reflex, with profound heart rate decrease secondary to a marked increased in vagal efferent discharge. To the best of our knowledge, dobutamine stress echocardiography unmasking clinically relevant sinus node dysfunction has never been reported. We report a case of a 50-year-old man presenting with chest pain and a history of syncope, who had worsening sinus bradycardia with isorhythmic AV dissociation during dobutamine stress echocardiography. An invasive electrophysiological study revealed marked sinus node dysfunction. The patient was referred for the insertion of a permanent pacemaker for sinus node dysfunction, chronotropic incompetence and symptomatic bradycardia. Dobutamine-associated bradycardia may not always be a benign peri-procedural physiologic effect, as in this case it may unmask clinically significant sinus node dysfunction.     

Fibrinolytic treatment with tissue plasminogen activator in a thrombosed modified Blalock-Taussig shunt]

Thrombolytic therapy of a systemic pulmonary fistula is a rare method of treatment in newborns with thrombosed systemic-pulmonary shunt. We report the case of a newborn girl with a complex congenital heart defect. On the ninth day of life a modified right Blalock-Taussig shunt was performed. The ductus arteriosus was not ligated. Six days later the baby developed severe hypoxemia. The results of echocardiography and cardiac catheterization showed a total thrombosis of the fistula and complete absence of flow throughout the shunt. After insertion of an arterial catheter into the proximal end of the shunt we started fibrinolytic treatment with recombinant tissue plasminogen activator (r-TPA) in continuous infusion. After 14 hours of treatment we confirmed by angiography complete clot dissolution. The baby left the hospital in good condition when she was 23 days old. In the follow-up (4th month of life) the shunt is still permeable. Thrombolytic therapy with r-TPA locally applied in case of acute thrombosis of a systemic-pulmonary shunt appears to be a good therapeutic option avoiding the risks of a new surgical procedure.