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Congenital Partial Pulmonary Venous Return Anomaly

TAPVC


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]
  • This case report illustrates an initial misleading clinical presentation of infracardiac TAPVR.[nature.com]
  • 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]
  • If an ASD is present,about 10% of patients will have a pulmonary venous abnormality. About 90% of sinus venosus defects and about 25% of ostium secundum defects present with PAPVR( 2, 3 ).[ispub.com]
Atrial Septal Defect
  • The patient was diagnosed as sinus venosus type of atrial septal defect with partial anomalous pulmonary venous return.[ispub.com]
  • The patient was diagnosed with sinus venosus type of atrial septal defect with partial anomalous pulmonary venous return.[heart.bmj.com]
  • 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]
  • Sinus venosus atrial septal defect association of this anomaly may be also seen.[omicsonline.org]
Falling
  • This may not always work, because in small children the amount of contast may be too small to trigger or due to breathing the cursor may fall to the lungs. If bolus tracking does not trigger, start the scan at 15 seconds.[radiologyassistant.nl]
  • Specific features of the surgical strategy are described depending on the level of confluence of the pulmonary veins, especially if the mouths of the abnormally falling pulmonary veins and the interatrial septum defect are far from each other.[rwjms1.umdnj.edu]
Hypertension
  • An echocardiogram showed severe pulmonary hypertension (right ventricle pressure of 88 mmHg).[nature.com]
  • 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]
  • 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]
  • Thus, the patients can be asymptomatic, present frequent colds—as in our case—or present heart failure and pulmonary hypertension.[archbronconeumol.org]
  • This anomaly is usually found in combination with hypoplasia of the right lung, pulmonary hypertension and other cardiac defects.[elsevier.pt]
Cyanosis
  • At 1 hour after birth, he presented cyanosis and mild tachypnea. An echocardiogram showed severe pulmonary hypertension (right ventricle pressure of 88 mmHg).[nature.com]
  • Pulmonary edema will lead to tachypnea and gasping, while mixing of blood supplies leads to cyanosis.[vhlab.umn.edu]
  • TAPVC can occur with obstruction, which occurs when the anomalous vein enters a vessel at an acute angle and can cause pulmonary venous hypertension and cyanosis because blood cannot enter the new vein as easily.[en.wikipedia.org]
Diastolic Murmur
  • Increased flow across the tricuspid valve may give rise to a diastolic murmur. Auscultation in the neonate may not reveal any murmurs if pulmonary blood flow is decreased due to elevated pulmonary vascular resistance.[rwjms1.umdnj.edu]

Workup

  • The workup includes TTE, TEE, and CTA.[lungindia.com]
  • CT is not part of the routine workup for FAI owing to concern about radiation exposure in young patients.[cdt-babes.ro]

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]
  • One umbilical artery and the umbilical vein were catheterized, and oxygen treatment was provided. Four echocardiograms indicated severe pulmonary hypertension and were negative for any congenital heart defects.[nature.com]

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]
  • 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]
  • […] 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]
  • 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]
  • 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]

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]
  • 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]
  • All attempts have been made to prevent disclosure of identity and personal information of the patient.[symbiosisonlinepublishing.com]

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