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| {{SI}} | | __NOTOC__ |
| | {{Partial anomalous pulmonary venous connection}} |
| | {{CMG}}; '''Associate Editor(s)-In-Chief:''' [[Priyamvada Singh|Priyamvada Singh, M.B.B.S.]] [mailto:psingh13579@gmail.com]; [[User:KeriShafer|Keri Shafer, M.D.]] [mailto:kshafer@bidmc.harvard.edu]; '''Assistant Editor(s)-In-Chief:''' [[Kristin Feeney|Kristin Feeney, B.S.]] [mailto:kfeeney@elon.edu] |
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| {{CMG}} | | {{SK}} PAPVC, isolated partial anomalous pulmonary venous connection |
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| '''Associate Editor-in-Chief:''' [[User:KeriShafer|Keri Shafer, M.D.]] [mailto:kshafer@bidmc.harvard.edu]
| | ==[[Partial anomalous pulmonary venous connection overview|Overview]]== |
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| {{Editor Join}}
| | ==[[Partial anomalous pulmonary venous connection anatomy|Anatomy]]== |
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| '''Synonyms and related keywords:''' PAPVC, isolated partial anomalous pulmonary venous connection
| | ==[[Partial anomalous pulmonary venous connection pathophysiology|Pathophysiology]]== |
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| ==Overview== | | ==[[Partial anomalous pulmonary venous connection epidemiology and demographics|Epidemiology and demographics]]== |
| In partial anomalous pulmonary venous connection (PAPVC) with intact atrial septum a portion (hence the term partial) of veins return to the [[right atrium]] rather than the [[left atrium]]. As a result oxygenated blood returning form the lung returns to the venous system via the [[right atrium]].
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| ==Epidemiology and Demographics== | | ==[[Partial anomalous pulmonary venous connection natural history|Natural History, Complications and Prognosis]]== |
| Autopsy data suggests that PAPVC has an incidence of 0.4-0.7%. Many of these autopsy cases were asymptomatic and as a result the clinical incidence of PAPVC is much lower.
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| ==Associated Conditions== | | ==[[Partial anomalous pulmonary venous connection causes|Causes]]== |
| PAPVC is present in approximately 10% of patients with an ASD. Depending upon the hemodynamics and magnitude and direction of shunting, an ASD may either improve or exacerbate the shunting associated with a PAPVC.
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| ==Differentiaion of PAPVC from Total Anomalous Pulmonary Venous Connection (TAPVC)== | | ==[[Partial anomalous pulmonary venous connection differential diagnosis|Differentiating Partial anomalous pulmonary venous connection from other Disorders]]== |
| These two defects have a common embryologic origin. However, in contrast to PAPVC, in TAPVC all [[pulmonary venous]] return is to the [[right atrium]].
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| ==Anatomy and Anatomic Variants==
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| Anatomically, PAPVC can involve a wide variety of connections, and PAPVC from the right lung is twice as common as PAPVC from the left lung. The most common form of PAPVC is one in which a right upper pulmonary vein connects to the right atrium or the superior vena cava (SVC). This form is almost always associated with a sinus venosus type of atrial septal defect (ASD).
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| In addition to draining into the right atrium, the anomalous right pulmonary veins can also drain into the inferior vena cava. Similarly, the left [[pulmonary vein]]s can drain into the [[innominate vein]], the [[coronary sinus]]. More rarely, the left pulmonary vein can drain into the cavae, the [[right atrium]], or the left [[subclavian vein]].
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| ==Pathophysiology==
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| The most important determinant of the ratio of pulmonary blood flow (Qp) to systemic flow (Qs) (the Qp/Qs ratio or the magnitude of the shunt) is the number of [[pulmonary veins]] that drain into the [[RA]]. The greater the number of [[pulmonary veins]] that drain into the [[RA}}, the greater the magnitude of the shunt. It has been speculated that when the proportion of returning [[pulmonary veins ]] that drain anomalously exceeds 50% of the pulmonary veins, then the anomaly is clinically significant. The position of the patient relative to the position of the anomalous pulmonary veins may also influence the magnitude of shunting. If a patient is standing, pulmonary blood flow is predominantly to the middle and the lower lobes of the lung. If there are a greater proportion of anomalous veins draining these portions of the lung, then standing can exacerbate the shunt. Other noncardiac conditions may influence the magnitude of shunting. One such disease state is the [[scimitar syndrome]] or pulmonary venolobar syndrome. In [[scimitar syndrome]] there is abnormal right-sided pulmonary venous drainage into the [[inferior vena cava]]. There is also malformation and/or hypoplasia of the right [[lung]] along with anomalous arterial flow to the right [[lung]].
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| ==Natural History and Complications==
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| There are few symptoms in infancy or childhood. After many years of excess [[pulmonary venous]] return, [[right atrial]] and [[right ventricular]] ([[RV]]) dilation may occur as a result of volume overload in the adult. [[RV]] dilation and enlargement is in turn associated with [[arrhythmias]], [[cor pulmonale]] or right-sided heart failure, and, in some patients, the development of [[pulmonary hypertension]].
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| ==Genetics==
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| There is no known genetic association.
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| ==Diagnosis== | | ==Diagnosis== |
| In general, children with partial anomalous pulmonary venous connection (PAPVC) are asymptomatic. Symptoms usually do not arise until adulthood following prolonged volume overload of the [[right ventricle]].
| | [[Partial anomalous pulmonary venous connection history and symptoms|History and Symptoms]] | [[Partial anomalous pulmonary venous connection physical examination|Physical Examination]] | [[Partial anomalous pulmonary venous connection laboratory tests|Laboratory Tests]] | [[Partial anomalous pulmonary venous connection electrocardiogram | Electrocardiogram]] | [[Partial anomalous pulmonary venous connection chest x ray|Chest X Ray]] | [[Partial anomalous pulmonary venous connection MRI|MRI]] | [[Partial anomalous pulmonary venous connection CT|CT]] | [[Partial anomalous pulmonary venous connection echocardiography|Echocardiography]] | [[Partial anomalous pulmonary venous connection other imaging findings|Other Imaging Findings]] | [[Partial anomalous pulmonary venous connection other diagnostic studies|Other Diagnostic Studies]] |
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| ===Symptoms=== | | ==Treatment== |
| *[[Palpitations]] are usually due to supraventricular [[arrhythmias]] which in turn are likely due to [[right atrial]] dilation.
| | '''Medical:''' [[Partial anomalous pulmonary venous connection medical therapy|Medical Therapy]] |
| *[[Dyspnea]] may develop in adults as a result of right ventricular volume overload and ensuing [[cor pulmonale]]. It has been said that [[dyspnea]] may occur earlier in children if over 50% of the [[pulmonary veins]] drain anomalously.
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| *[[Hemoptysis]] may reflect the development of [[pulmonary hypertension]] and is rare.
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| *[[Pedal edema]] can occur as a result of [[right ventricular failure]] or [[cor pulmonale]].
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| ===Cardiac Magnetic Resonance (CMR)===
| | '''Surgical:''' [[Partial anomalous pulmonary venous connection surgery|Surgery]] |
| CMR is the imaging modality of choice in diagnosis and evaluating the structure of PAPVC. Other imaging modalities such as echocardiography may diagnose the presence of PAPVC, but CMR may yiled valuable insight into the number and location of pulmonary veins that are anomalously draining into the [[right atrium]].
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| ===Chest X Ray===
| | [[Partial anomalous pulmonary venous connection primary prevention|Primary Prevention]] | [[Partial anomalous pulmonary venous connection secondary prevention|Secondary Prevention]] | [[Partial anomalous pulmonary venous connection cost-effectiveness of therapy|Cost-Effectiveness of Therapy]] | [[Partial anomalous pulmonary venous connection future or investigational therapies|Future or Investigational Therapies]] |
| [[Cardiomegaly]] and increased vascular markings may be observed on chest x ray. The main [[pulmonary artery]] may be dilated. The [[mediastinum]] may be widened due to veins draining from the left [[lung]] into the left vertical vein. | |
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| There may be evidence of the [[Scimitar syndrome]] in which an anomalous vein drains into the [[inferior vena cava]] at the level of the diaphragm.
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| It should be noted that the chest x ray may be normal.
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| ===Echocardiographic Findings===
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| The presence of [[right ventricular]] dilation may be the first sign that PAPVC is present. The next step is to visualize all the major pulmonary veins and their points of insertion into the heart. The use of agitated saline and contrast echocardiography can be of help in more refined imaging of the venous architecture and the connections to the heart. When agitated saline is injected in the left arm vein, there may be negative contrast in the innominate vein at the side of the anomalous venous drainage from a left pulmonary vein in patients with PAPVC. Transesophageal echocardiography may also be useful in further imaging the venous structures. Becuase PAPVC is associated with ASDs, the interatrial septum should be carefully evaluated.
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| ===Computed Tomography===
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| If echocardiographic visualization of structures is non diagnostic, then contrast-enhanced CT is an alternative imaging modality.
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| ==References== | | ==References== |
| {{reflist|2}} | | {{reflist|2}} |
| | | {{WH}} |
| {{Congenital malformations and deformations of circulatory system}} | | {{WS}} |
| {{SIB}} | |
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| [[Category:Cardiology]] | | [[Category:Cardiology]] |
| [[Category:Congenital heart disease]] | | [[Category:Congenital heart disease]] |
| | | [[Category:Pediatrics]] |
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