Presentation
The time is definitely right for a comprehensive presentation of these new diagnostic modalities and the way in which they will influence the field of congenital heart management. * First and only volume on the market presenting all the pre- and post-operative [books.google.com]
Case presentation A 48-year-old former smoker presented with a 6-month history of cough and blood sputum. [jtd.amegroups.com]
Patients more frequently present with symptoms when associated anomalies are present and may require repair using cardiopulmonary bypass [7]. [ctsnet.org]
In addition, frequently present is an abnormal right pulmonary arterial supply consisting of aortopulmonary collaterals. Adults often present with dyspnea and recurrent pulmonary infections. [annals.in]
PAPVR may occasionally present as an incidental finding in an asymptomatic patient. Symptomatic patients usually present with a spectrum of symptoms including supraventricular tachycardia, exertional dyspnea, and chronic fatigue. [massgeneral.org]
Entire Body System
- Atrial Septal Defect
Surgical repair of atrial septal defect with partial anomalous pulmonary venous return include provision of durably unobstructed systemic and pulmonary venous pathways, closure of the atrial septal defect, and avoidance of arrhythmias. [link.springer.com]
The patient was diagnosed with sinus venosus type of atrial septal defect with partial anomalous pulmonary venous return. [heart.bmj.com]
Sinus venosus atrial septal defect association of this anomaly may be also seen. [omicsonline.org]
It is frequently associated with other congenital heart disease, most commonly atrial septal defect. The overall incidence is estimated to be between 0.4%-0.7%. [casereports.in]
Gotsman MS, Astley R, Parsons CG: Partial anomalous pulmonary venous drainage in association with atrial septal defect. Br Heart J 1965;27:566–571. [karger.com]
- Asymptomatic
Sometimes asymptomatic congenital venous anomalies may be brought into clinical light by malpositioning incidence. [symbiosisonlinepublishing.com]
A cardiac magnetic resonance scan was performed on a 32-year old asymptomatic male referred for further evaluation of cardiac murmur. [casereports.in]
Her history was notable for hypertension, hyperlipidemia, asymptomatic coronary artery disease, and a colectomy secondary to diverticulitis. A transthoracic cardiac ultrasound (TTE) revealed no evidence of significant structural heart disease. [massgeneral.org]
Technique Indications Operative intervention has been recommended for A) the presence of symptoms or B) in an asymptomatic patient with a pulmonary to systemic flow ratio (Qp:Qs) greater than 1.5:1 or enlargement of right heart chambers, to decrease the [ctsnet.org]
PAPVR is traditionally associated with atrial septal defects, and patients are often asymptomatic. Clinical manifestations occur when there is significant shunting and include syncope, right heart failure, and pulmonary hypertension (2). [swjpcc.com]
- Pain
History A 55-year-old male patient (heart rate 75 bpm, body mass index 26.0 kg/sqm) suffered from progredient physical inefficiency and atypical chest pain since several months. [dsct.com]
Clinically malposition should be suspected when there is poor backflow in ports, resistance to infusion, infusion related chest pain, arm pain, head ache, gurgling sound in ear [3]. [symbiosisonlinepublishing.com]
Patients with large shunts may present with symptoms of dyspnea, chest pain and palpitations, signs like tachycardia and murmur can be encountered. Cases of secondary pulmonary arterial hypertension have been reported 6,7. [radiopaedia.org]
In the ER you will see these patients because they age and get chest pain like many adults do and so you will see these anomalies more frequently. [radiologyassistant.nl]
- Surgical Procedure
If removal carries risk of bleeding from puncture point which cannot be compressed externally endovascular or surgical procedure may have to be resorted to [3]. This study involved human subject participation. [symbiosisonlinepublishing.com]
Learning objective: To give information about the possible surgical technique in adult congenital patient with anomalous pulmonary veins connection who had previously undergone surgical procedure. © 2015 Published by Elsevier Sp. z o.o. on behalf of the [e-coretvasa.cz]
- Recurrent Infection
infections. [3] Therapeutic options for the repair of PAPVC vary with the type of anomaly [10] and coexistence of other cardiac defects. [lungindia.com]
Respiratoric
- Dyspnea
She complained of dry cough and dyspnea that had persisted during the previous one month. [synapse.koreamed.org]
The patient was symptom-limited to significant dyspnea on exertion. He was able to walk 150 m in the 6-min walk distance. [lungindia.com]
These include dyspnea (breathlessness) in response to exertion, atrial arrhythmias (flutter or fibrillations), or, more rarely, right heart failure and pulmonary hypertension. [pted.org]
Our case has dyspnea, palpitation and arrhythmia, and his right heart cavities were mildly dilated. [omicsonline.org]
Patients with large shunts may present with symptoms of dyspnea, chest pain and palpitations, signs like tachycardia and murmur can be encountered. Cases of secondary pulmonary arterial hypertension have been reported 6,7. [radiopaedia.org]
- Exertional Dyspnea
Symptomatic patients usually present with a spectrum of symptoms including supraventricular tachycardia, exertional dyspnea, and chronic fatigue. [massgeneral.org]
A 19 - year- old woman presented to our hospital with exertional dyspnea and sinus tachycardia.Her chest X-ray demonstrated cardiomegaly with prominent right atrium, right ventricle and dilated pulmonary vessels (Figure 1). [ispub.com]
Case presentation A 38-year-old woman from Elazig, Turkey with atrial septal defect was admitted for exertional dyspnea and fatigue. She had a general tendency and swelling both of the lower limbs at term (38-40 weeks' gestation). [link.springer.com]
The patient was symptom-limited to significant dyspnea on exertion. He was able to walk 150 m in the 6-min walk distance. [lungindia.com]
Cardiovascular
- Heart Disease
NIH: National Heart, Lung, and Blood Institute Atrial septal defect (Medical Encyclopedia) Bicuspid aortic valve (Medical Encyclopedia) Congenital heart defect corrective surgeries (Medical Encyclopedia) Congenital heart disease (Medical Encyclopedia) [icdlist.com]
TAPVR; Total veins; Congenital heart defect - TAPVR; Cyanotic heart disease - TAPVR Fraser CD, Kane LC. Congenital heart disease. In: Townsend CM Jr, Beauchamp RD, Evers BM, Mattox KL, eds. [medlineplus.gov]
It demonstrates that noncyanotic adult congenital heart diseases can be associated with nocturnal hypoxemia. [lungindia.com]
Friedman W, Congenital heart disease in infancy and childhood, in Heart Disease: A Textbook of Cardiovascular Medicine, E. Braunwald, Editor. 1992, WB Saunders: Philadelphia. pp887–965. [ctsnet.org]
disease, 040293, *, 奇形, キケイ, abnormality, 012757, 先天性疾患, センテンセイシッカン, congenital disease, 040292, 病気, ビョウキ, disease, 017213, 心臓病, シンゾウビョウ, heart disease, 023405, 心血管疾患, シンケッカンシッカン, cardiovascular disease, 023404, 動静脈奇形, ドウジョウミャクキケイ, arteriovenous [togodb.biosciencedbc.jp]
- Hypertension
Pulmonary hypertension may also occur and arrhythmias are sometimes experienced after surgery - a situation that increases in likelihood with the age at repair. [pted.org]
Patients with PAPVC in the long term can develop pulmonary hypertension. [9] Rafanan et al. [13] have also reported nocturnal desaturation in patients with pulmonary arterial hypertension. [lungindia.com]
An echocardiogram showed severe pulmonary hypertension (right ventricle pressure of 88 mmHg). [nature.com]
Thus, the patients can be asymptomatic, present frequent colds—as in our case—or present heart failure and pulmonary hypertension. [archbronconeumol.org]
In the majority of patients, surgical correction is curative, with no further evidence for pulmonary hypertension or recurrent pulmonary venous obstruction. [clinicaladvisor.com]
- Heart Failure
These include dyspnea (breathlessness) in response to exertion, atrial arrhythmias (flutter or fibrillations), or, more rarely, right heart failure and pulmonary hypertension. [pted.org]
If this condition is not treated, the heart will get larger, leading to heart failure. Repairing the defect early provides excellent results if there is no blockage of the pulmonary veins at the new connection into the heart. [medlineplus.gov]
Clinical manifestations occur when there is significant shunting and include syncope, right heart failure, and pulmonary hypertension (2). [swjpcc.com]
Thus, the patients can be asymptomatic, present frequent colds—as in our case—or present heart failure and pulmonary hypertension. [archbronconeumol.org]
When a vascular shunt is present, major lung resection may cause acute right heart failure. [jtd.amegroups.com]
- Heart Murmur
Adult patients who have not been operated on in childhood may need to undergo repair if right heart enlargement (dilalation) occurs. This will be recognized by a heart murmur or by echocardiography. [pted.org]
Cyanotic heart disease (Medical Encyclopedia) Dextrocardia (Medical Encyclopedia) Echocardiogram -- children (Medical Encyclopedia) Heart murmurs and other sounds (Medical Encyclopedia) Patent ductus arteriosus (Medical Encyclopedia) Ventricular septal [icdlist.com]
Infants with TAPVR or other conditions causing cyanosis can have symptoms such as: Problems breathing Pounding heart Weak pulse Ashen or bluish skin color Poor feeding Extreme sleepiness Using a stethoscope, a doctor will often hear a heart murmur (an [cdc.gov]
The diagnosis of total anomalous pulmonary venous return may be initially suspected when a physician hears a typical heart murmur and detects evidence of overload on the right side of the heart. [cincinnatichildrens.org]
Signs of TAPVR include a bluish tint to lips, nails and skin, rapid breathing and sometimes a heart murmur – or whooshing sound – heard via a stethoscope. [childrens.memorialhermann.org]
- Cardiomegaly
Cardiomegaly can also be seen if significant abnormal intracardiac venous drainage occurs. CT Utilization of contrast-enhanced studies with MDCT technology enables both detection and characterization of the anomalies. [radiopaedia.org]
There is cardiomegaly with increased pulmonary arterial markings. There is dilation of both the left and right innominate veins and the right superior vena cava producing the classical "snowman" or "figure of 8" appearance. [bcm.edu]
A 19 - year- old woman presented to our hospital with exertional dyspnea and sinus tachycardia.Her chest X-ray demonstrated cardiomegaly with prominent right atrium, right ventricle and dilated pulmonary vessels (Figure 1). [ispub.com]
Signs and symptoms [ edit ] right ventricular heave Loud S1 fixed split S2 S3 gallop systolic ejection murmur at left upper sternal border cardiomegaly right axis deviation on ECG Snowman sign or `figure of 8 configuration` on chest radiograph right ventricular [en.wikipedia.org]
The chest X-ray ( Figure 3 A) showed cardiomegaly and moderate pulmonary venous congestion. [elsevier.pt]
Workup
The workup includes TTE, TEE, and CTA. [lungindia.com]
Treatment
Many children with congenital heart defects don't need treatment, but others do. Treatment can include medicines, catheter procedures, surgery, and heart transplants. [icdlist.com]
The management of PH with ILD includes the treatment of PH itself, and the underlying ILD ( 6 ). Standard PH treatment (e.g vasomodulating agent) is a major component of various treatment options. [synapse.koreamed.org]
But the patient did not accept either surgical or medical treatment and no further evaluation could be performed. [omicsonline.org]
Ongoing controversies regarding etiology, diagnosis, treatment The etiology is incompletely understood, and there are many theories. Diagnosis and treatment are reasonably incontrovertible. [clinicaladvisor.com]
Treatment The operation to repair PAPVR is performed under general anesthesia, which means you will be asleep during surgery. First, the surgeon makes a vertical incision in the front of the chest, opens the breastbone, and exposes the heart. [uwhealth.org]
Prognosis
The prognosis for surgically corrected non-scimitar PAPVR is excellent. Scimitar syndrome PAPVR has a less favorable prognosis due to other associated cardiac and pulmonary lesions. [indiana.pure.elsevier.com]
Treatment and prognosis Therapeutic options include surgical repair with ASD patching, intracardiac baffle, anomalous vein anastomosis, systemic vein translocation and Warden procedure inter alia. [radiopaedia.org]
[…] human, 肺静脈還流異常, ハイジョウミャクカンリュウイジョウ, anomalous pulmonary venous return, 057874, *, 手術手技, シュジュツシュギ, surgical technique, 060856, 心臓血管外科, シンゾウケッカンゲカ, cardiovascular surgery, 055961, 術後合併症, ジュツゴガッペイショウ, postoperative complication, 054363, 予後, ヨゴ, prognosis [togodb.biosciencedbc.jp]
Generally, patients with PH and associated ILD are known to have a worse prognosis ( 2, 13 ). When a patient with PH and ILD shows signs of worsening dyspnea, pure PH deterioration, ILD progression and pulmonary infection are commonly assessed. [synapse.koreamed.org]
The prognosis after surgery during childhood is excellent with uncommon late complications. Rarely, obstruction of the pulmonary veins at the site of reconnection can occur. Occasionally abnormal heart rhythms can also occur. [heart.org]
Etiology
Ongoing controversies regarding etiology, diagnosis, treatment The etiology is incompletely understood, and there are many theories. Diagnosis and treatment are reasonably incontrovertible. [clinicaladvisor.com]
Echocardiography The etiology of PAPVR is not that well understood. Why do one or more pulmonary veins deviate and attach to another central venous structure? They may attach to any of the systemic venous vessels. [heartdefectssimplified.com]
Another possible etiology of nocturnal hypoxemia is obstructive sleep apnea (OSA). Although OSA has been known to be associated with heart failure, [15] resolution of the hypoxemia after surgery makes this diagnosis less likely. [lungindia.com]
Physicians who diagnose and treat adult patients with pulmonary hypertension, right chamber dilatation with signs of volume overload and an intact atrial septum should always consider abnormal venous pulmonary drainage as a potential etiology. [elsevier.pt]
Pathophysiology
(Click picture to show/hide bloodflows) Pathophysiology Patients with partial anomalous pulmonary venous return are usually asymptomatic. [vhlab.umn.edu]
Alpert JS, Dexter L, Vieweg WVR, Haynes FW, Dalen JE: Anomalous pulmonary venous return with intact atrial septum: Diagnosis and pathophysiology. Circulation 1977;56:870–875. [karger.com]
The high prevalence of atrial arrhythmias in patients with surgically repaired CHD can be due to diffuse pathophysiologic changes of atrial muscles, progressive atrial myopathy due to chronic volume overload of the RA, or right atriotomy or other incisional [innovationsincrm.com]
We discuss the pathophysiologic mechanisms that may lead to nocturnal hypoxemia and postulate the rationale behind its resolution. A 65-year-old man presented to our clinic with dyspnea and swelling of both the legs for 6 months. [lungindia.com]
Alpert JS, Dexter L, Vieweq WV, et al Anomalous pulmonary venous return with intact atrial septum: diagnosis and pathophysiology. Circulation 1977; 56:870–875. 21. [journals.lww.com]
Prevention
What about preventing endocarditis? Children with TAPVC are at increased risk for developing endocarditis. Ask your pediatric cardiologist about your child's need to take antibiotics before certain dental procedures to help prevent endocarditis. [heart.org]
Children’s heart conditions can’t be prevented, but a lot can be done to improve and often completely repair their hearts at any age. [nemours.org]
All attempts have been made to prevent disclosure of identity and personal information of the patient. [symbiosisonlinepublishing.com]
As a matter of courtesy we request that the content provider (Centers for Disease Control and Prevention, National Center on Birth Defects and Developmental Disabilities) be credited and notified in any public or private usage of this image. [cdc.gov]
ASDs should be closed with a patch, and anomalous vessels as well as the ductus arteriosus ligated to prevent left-to-right shunts. [vhlab.umn.edu]