Ventricular Septal Defect (VSD)
Cardiac catheterization
Cardiac catheterization to assess the feasibility of surgery in adult patients with VSD combined with pulmonary arterial hypertension (PAH) should be performed in collaboration with appropriate specialists at the regional adult congenital heart disease (ACHD) center (Class I recommendation, Level of Evidence: C, abbreviated I/C).
Cardiac catheterization is valid for adult patients with VSD in cases where noninvasive findings are inconclusive and treatment requires more information. The following data should be collected during the examination:
1. Fractional flow at the defect (IIa/B);
2. Measurement of pulmonary artery pressure and resistance in patients with suspected PAH, and determination of PAH reversibility with various vasodilators (IIa/B);
3. Evaluate other cardiac lesions, such as aortic regurgitation and double chamber right ventricle (IIa/C);
4. Determine the presence of multiple VSD (IIa/C) prior to surgery;
5. Patients at risk for coronary artery disease should undergo coronary angiography (IIa/C);
6. The anatomy of the VSD should be understood, especially before the proposed application of the occlusion device (IIa/C).
Treatment strategy
Drug therapy
● Adult patients with VSD in combination with progressive or severe pulmonary vascular disease can be treated with pulmonary artery dilators (IIb/B).
Surgical closure of VSD
● Trained surgeons and CHD specialists can perform VSD closure surgery (I/C).
● VSD closure should be performed in patients with a pulmonary circulation to body circulation flow ratio (Qp/Qs) ≥ 2.0 and clinically proven left ventricular volume overload (I/B).
● Patients with a history of infective endocarditis may undergo VSD closure (Ⅰ/C).
Patients with pulmonary artery pressure less than 2/3 of systemic blood pressure, pulmonary vascular resistance less than 2/3 of systemic vascular resistance, Qp/Qs >1.5, and a simple left-to-right shunt may receive a closed VSD (IIa
/B).
For patients with left ventricular systolic or diastolic failure, Qp/Qs >1.5 and a pure left-to-right shunt, closed VSD (IIa/B) is indicated.
In patients with severe irreversible PAH, closed VSD (III/B) should not be performed.
Catheter intervention
Closure of myocardial VSDs with a blocking device should be done with caution, especially for defects away from the tricuspid valve and aorta, or for VSDs (IIb/C) in combination with severe enlargement of the left heart chambers or PAH.’
Evaluation and follow-up
Follow-up after surgical and interventional interventions
● Adult patients with residual heart failure, shunts, PAH, aortic regurgitation, or left or right ventricular outflow tract obstruction should be examined at the regional ACHD center at least once a year after VSD (I/C).
Adult patients with residual mild VSD and no other co-morbidities should be examined at their regional ACHD center every 3 to 5 years (I/C).
Adult patients with a blocking device should be followed up at an ACHD center every 1 to 2 years, depending on the location of the VSD and other factors (I/C).
Pregnancy
Pregnancy is not recommended in women with ASD with severe PAH (Eisenmenger syndrome) because of the high maternal and fetal mortality rates, and should be strongly discouraged by the physician (III/A).
Atrial septal defect (ASD)
Evaluation of the non-operated patient
The diagnosis of ASD should be made with evidence of shunt at the defect, right ventricular volume overload, and any associated anomalies on imaging (I/C).
●
Patients with unexplained right ventricular volume overload should be referred to an ACHD center for further diagnostic testing to rule out cryptogenic ASD, partial pulmonary vein ectopic connections, or coronary sinus septal defect (I/C).
For patients with mild to moderate PAH whose symptoms do not match the clinical findings or whose oxygen saturation has changed, a maximum load exercise test may be given to determine their mobility (IIa/C).
● Combined coronary artery disease due to age or other risk factors can be excluded by cardiac catheterization (IIa/B).
● Diagnostic cardiac catheterization is not necessary in young patients with uncomplicated ASD who have clear imaging studies (III/B).
● Maximum load exercise test (III/B) is not recommended for patients with ASD combined with severe PAH.
Treatment strategy
Drug therapy
If the patient has atrial fibrillation, cardioversion should be performed to restore sinus rhythm after appropriate anticoagulation therapy (I/A).
If the patient is unable to maintain sinus rhythm by pharmacological or interventional means, ventricular rate control and anticoagulation therapy are recommended (I/A).
Interventional and surgical treatment
Patients with right atrial and right ventricular enlargement with or without symptoms should undergo percutaneous or surgical repair of ASD (I/B).
For venous sinus, coronary sinus, or foramen ovale ASDs, surgical repair rather than percutaneous closure should be given (I/B).
Trained surgeons and CHD specialists can perform different types of ASD closure procedures (I/C).
Surgical closure of secondary foramen ovale ASDs (IIa/C) may be considered when the patient is also undergoing tricuspid valve repair (replacement) or when the anatomy of the defect site is not suitable for a percutaneous device.
● When paradoxical embolism (IIa/C) or upright hypoxemia-oblique breathing (IIa/B) is present, ASD should be closed by intervention or surgery.
Patients with a simple left-to-right shunt, pulmonary artery pressure less than 2/3 of systemic blood pressure, pulmonary vascular resistance less than 2/3 of systemic vascular resistance, or patients who respond to pulmonary vasodilator therapy or defect closure tests should be considered for interventional or surgical closure of the ASD (treatment should be performed in collaboration with a physician experienced in the treatment of pulmonary hypertension syndrome) (IIb/C).
In adult patients with ASD combined with paroxysmal or chronic atrial tachycardia, concurrent Maze surgery may be considered (IIb /C).
Patients with severe irreversible PAH and no evidence of left-to-right shunt should not be closed ASD (III/B).
Follow-up after interventional therapy
● Excessive fever, fatigue, vomiting, chest pain, or abdominal pain in the early postoperative period may be associated with pericardial tamponade due to postoperative pericardiotomy syndrome, and patients should undergo prompt cardiac ultrasonography (I/C).
Adult patients with ASD who have persistent or occurring ASD should receive annual clinical follow-up after surgery (I/C):
1. PAH,
2. Atrial arrhythmias,
3. right or left ventricular dysfunction,
4. Combined valvular or other cardiac lesions.
Patients 3 months to 1 year after blocking should be examined for blocker displacement, wear, or other complications and followed up periodically (I/C).
● Patients with worn blocking devices may present with chest pain or fainting, and should be examined immediately (Ⅰ/C).
Pregnancy is not recommended in women with ASD with severe PAH (Eisenmenger syndrome) because of the high maternal and fetal mortality rates, and should be strongly discouraged by the physician (III/A).
Table Evaluation and monitoring of adult atrial septal defects
Before treatment
Symptoms
Respiratory distress
Weakness
Impaired exercise tolerance
Heart palpitations
Syncope
Shunt size
Echocardiography shows right ventricular volume over
overload
Chest x-ray showing pulmonary congestion
Defect size, site, valve margin
secondary orifice
primary orifice
venous sinus
coronary sinus
Associated lesions
mitral valve dehiscence
Pulmonary valve stenosis
Pulmonary vein anomaly
mitral valve prolapse
Permanent left superior vena cava
Coronary artery related diseases
pulmonary artery pressure
Tricuspid valve by echocardiography
regurgitant flow to assess
Systolic valve pressure flattening
Arrhythmias
Atrial fibrillation
Atrial flutter
Paroxysmal atrial tachycardia
Morbid sinus node syndrome
Heart block
Paradoxical emboli, elimination
Venous stasis
Venous access without filtering device
Indwelling catheter
Post-treatment
After surgical treatment
Pericardial effusion/constriction
Residual shunt
Right ventricular systolic and diastolic dysfunction
Pulmonary artery pressure
Mitral regurgitation
Pulmonary vein stenosis or vena cava stenosis
(venous sinus defect)
Cardiac arrhythmia
Tricuspid regurgitation
After catheterization
Device maladjustment
Device embolism
Atrial wall or aortic device wear
Device encroachment on adjacent structures
● Atrioventricular valve
● Coronary sinus
● Superior vena cava
● Pulmonary vein
● Aorta
Device thrombosis
Endocarditis in the first 6 months after surgery
or with residual defect
Residual shunt
Evaluation of patients with patent ductus arteriosus (PDA) who have not undergone surgery
● Definitive diagnosis of PDA may be based on shunts at the defect (with or without clinical manifestations of significant left ventricular volume overload) as demonstrated by imaging (Class I recommendation, Level of Evidence: C, referred to as I/C).
● Patients with uncomplicated PDA clearly diagnosed by appropriate noninvasive imaging should not undergo diagnostic cardiac catheterization (III/B).
● Patients with PDA combined with significant pulmonary arterial hypertension (PAH) should not be given a maximal load exercise test (III/B).
Closure of the arterial catheter
● Percutaneous or surgical closure of the arterial catheter (I/C) may be performed when the patient has
1. Enlarged left atrium and/or left ventricle, combined PAH or simple left-to-right shunt;
2. Previous history of endarteritis.
● Patients with PDA with calcification should consult an adult congenital heart disease (CHD) interventional specialist (I/C) before opting for surgical closure.
● Surgery should be performed by a surgeon experienced in the treatment of CHD when the patient has the following conditions (I/C).
1. The PDA is larger than the closure device;
2. The catheter anatomy is distorted and the closure device cannot be applied.
● Interventional catheters can be used to close small asymptomatic PDAs (IIa /C).
● For PDA with combined PAH and simple left-to-right shunt, it can be closed (IIa /C).
● PDA with combined PAH and simple right-to-left shunt should not be closed (III/C).
Internal treatment
● Patients with small PDA without left heart volume overload should receive routine follow-up; patients with small PDA with left heart volume overload should be followed up every 3 to 5 years (I/C).
● Patients with PDA who have undergone repair without residual shunts do not need endocarditis prophylaxis (III/C).
Atrioventricular septal defect (AVSD)
Cardiac catheterization
● Cardiac catheterization can be used to evaluate patients for PAH and to test their vascular response in patients with AVSD who have or have not undergone repair (IIa/B).
Surgical treatment
● Trained surgeons and CHD specialists can perform AVSD closure procedures (I/C).
● Adult patients with AVSD who have undergone repair should undergo a repeat procedure (I/B) if
1. left atrioventricular valve repair or replacement is recommended when clinical symptoms of left atrioventricular valve stenosis or insufficiency of closure, atrial or ventricular arrhythmias, progressive increase in left ventricular diameter, or deterioration of left ventricular function occur;
2. Left ventricular outflow tract obstruction with a mean pressure step >50 mmHg, or a peak instantaneous pressure step >70 mmHg, or a pressure step <50
mmHg but combined with significant mitral or aortic regurgitation;
3. Residual or recurrent atrial septal defect or ventricular septal defect with significant left-to-right shunt.
Prevention of endocarditis
● Patients at high risk for CHD with poor prognosis of infective endocarditis (IE) should receive prophylactic antibiotics before undergoing dentistry involving gingival or periapical dental tissue manipulation or oral mucosal puncture. This includes the following conditions (IIa/
B).
1, Heart valve repair using a prosthetic heart valve or prosthetic material;
2. History of infective endocarditis;
3, Cyanotic CHD without repair or palliative care, including surgically established palliative shunts and artificial tubes;
4, CHD completely repaired by surgical or interventional methods with artificial materials within 6 months after surgery;
5. CHD that has been repaired but still has residual defects at or near the artificial patch or blocking device that inhibit endothelialization of the artificial material.
● For high-risk female patients with poor prognosis, antibiotics may be considered for the prophylaxis of IE when the membranes are ruptured by transvaginal delivery. indications are as follows (IIa/ C)
1, Those with heart valve repair using an artificial heart valve or prosthetic material;
2. Cyanotic CHD without repair or palliative care, including surgically established palliative shunts and artificial tubes.
● Patients without active infection undergoing non-dental procedures (e.g., fiberoptic gastroscopy or colonoscopy) should not be given antibiotics for IE prophylaxis (III/C).
Pregnancy
● Obvious residual hemodynamic lesions can complicate management during pregnancy, so all female patients with a previous history of AVSD should be evaluated for these conditions before preparing for pregnancy (I/C).
● Pregnancy risks and contraceptive measures should be discussed with female patients with Down syndrome and their guardians (I/C).