[의문] 선천성 심장 결함 : 청색증과 동맥관 의존성

Common critical congenital heart defects and their association with cyanosis and dependence upon the ductus arteriosus

	Cyanosis ?	Ductal-dependent ?
Left-sided obstructive lesions
Hypoplastic left heart syndrome	O	O
Valvar AS
Critical AS	Cyanosis or differential cyanosis*	O
Moderate to severe AS	X	X
COA
Critical COA	Differential cyanosis*	O
Moderate to severe COA	X	X
Interrupted aortic arch	Differential cyanosis* (pattern of cyanosis varies based upon type)	O
Right-sided obstructive lesions
Tetralogy of Fallot	Variable	Δ ¶
Tetralogy of Fallot with pulmonary atresia	O	O (unless multiple or large aortopulmonary collaterals are present)
Pulmonary atresia with intact interventricular septum	O	O
PS
Critical PS	O	O
Severe PS	X	X
Tricuspid atresia	O	Δ ¶
Severe neonatal Ebstein anomaly	O	Δ #
Parallel circulations
Transposition of the great arteries	O ◊	O
Other
TAPVC	O	X §
Large VSD	X	X
AV canal defect	X	X
Truncus arteriosus	O	X

REF : UPTODATE 15.09.2023

Abbreviation & Sign

AS: aortic stenosis; COA: coarctation of the aorta; PS: pulmonic stenosis; TAPVC: total anomalous pulmonary venous connection; VSD: ventricular septal defect; AV: atrioventricular; CHD: congenital heart disease; RV: right ventricle; PDA: patent ductus arteriosus.

* In these lesions, the upper one-half of the body (preductal) is pink and the lower one-half (postductal) is cyanotic.
¶ Infants with tetralogy of Fallot or tricuspid atresia may have ductal-dependent circulation if there is severe RV outflow tract obstruction (ie, critical pulmonary stenosis or atresia).
# In cases of severe Ebstein anomaly with extreme cyanosis, a PDA may be necessary to maintain pulmonary blood flow until pulmonary vascular resistance drops.
◊ Reversed differential cyanosis (ie, oxygen saturation higher in the lower than upper extremity) may occur if there is coexisting coarctation of the aorta or pulmonary artery hypertension.
§ Some patients with obstructed TAPVC may require a PDA to maintain systemic cardiac output. However, a PDA may also increase the degree of cyanosis.

 

This information has been obtained from UPTODATE. (It will be deleted or made private if it becomes a problem.)

This material is a reorganization of content extracted from textbooks or various problem sets, with an emphasis on cyanosis and ductal dependency. 
However, without prior knowledge of existing diseases, one might overlook the significance of ductal dependency.
The term "ductal dependency" can refer to the worsening of symptoms when the ductus arteriosus closes completely at around 1-2 weeks of life, and it may also indicate an association between the disease and the patency of the ductus arteriosus.

In section §, it is mentioned that some patients with obstructed TAPVC may require a patent ductus arteriosus (PDA) to maintain systemic cardiac output. However, a PDA may also increase the degree of cyanosis. It raises the question of whether TAPVC can be considered ductally dependent, but it is noted as "X(No)" regarding ductal dependency.

On the other hand, regarding cyanosis, it should be relatively easy to determine whether it occurs depending on the disease.

Furthermore, from the material, it appears that ductal dependency and cyanosis often coexist. Considering the anatomical location of the ductus arteriosus, one might think that cyanosis is not necessarily inevitable when the ductus arteriosus remains patent.

However, if one considers that diseases worsen when the ductus arteriosus closes, this logic doesn't hold. Therefore, it would be beneficial to distinguish diseases that cause cyanosis from those that do not and study the relationship between diseases and the patency of the ductus arteriosus in depth.

 

If anyone would like to share their opinions, please leave a comment.

 

 

 

 

해당 자료는 UPTODATE에서 가져왔습니다. (문제가 될 시에는 삭제하거나 비공개로 전환할 예정)

 

이 자료는 교과서나 각 문제집에 정리된 내용을 청색증과 동맥관 의존성으로 재구성한 내용으로 의미가 있습니다.

하지만 기존 질병에 대한 지식이 없다면 동맥관 의존성 (Ductal-dependent) 이 의미하는 바를 놓칠 수 있다고 생각합니다. 

동맥관 의존성이라는 뜻은 동맥관이 완전히 폐쇄되는 생후 1-2주 경에 증상이 악화되는 것을 두고 동맥관 의존성이라고 표현할 수도 있고, 또한 질병과 동맥관 개존과의 연관성이 있음을 두고 동맥관 의존성이라고 표현할 수도 있다고 보여집니다. 

 

기호 §를 보면, Some patients with obstructed TAPVC may require a PDA to maintain systemic cardiac output. However, a PDA may also increase the degree of cyanosis. TAPVC의 경우에는 체순환을 유지하기 위해서 동맥관의 개존을 요구한다. 반면에 동맥관 개존은 청색증을 유발한다라고 되어있습니다. 이 경우에는 TAPVC가 동맥관 의존성을 지닌다고 볼 수 있나 했더니 동맥관 의존성에는 X(No)라고 표기하고 있습니다.

 

 

반면에 청색증의 경우에는 질병에 따라서 청색증이 나타나는지 아닌지에 관한 내용임을 쉽게 파악할 수 있을 것이라고 생각합니다. 

 

게다가 자료를 보면, 동맥관 의존성과 청색증이 함께 하는 것처럼 보입니다. 동맥관의 해부학적 위치를 고려할 때에 동맥관이 개존된 상태에서 청색증이 나타나는 것이 필연적이지 않나-라고 생각할 수도 있겠죠. 하지만 여기서 동맥관이 폐쇄되는 시점에 질병이 악화된다고 생각하면 이러한 논리는 맞지 않습니다. 따라서 청색증이 나타나는 질병과 그렇지 않은 질병으로 구분하고, 동맥관과의 질병의 관계에 대해서는 심도있게 공부를 하는 편이 좋을 것 같습니다. 

 

 

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