Echocardiographic assessment in Adult Patients with Congenital Heart Diseases

Transcription

1 Echocardiographic assessment in Adult Patients with Congenital Heart Diseases Athanasios Koutsakis Cardiologist, Cl. Research Fellow George Giannakoulas Ass. Professor in Cardiology 1st Cardiology Department, Aristotle University of Thessaloniki Ιπποκράτειες ημέρες καρδιολογίας Thessaloniki, 09 March 2018

2 None Declaration of interest

3 Epidemiology of CHD Total CHD birth prevalence: 9 per 1,000 live births 1.35 million newborns with CHD every year A major global health burden

4 Survival to 18 years of age with CHD Decade Born with CHD % Survival Percent Survival to 18 Years Old Warnes CA, et al. J Am Coll Cardiol. 2001;37(5): Moons P et al. Circulation :

5 Improved Surgical Techniques Fetal Diagnosis Expanding Population of Adolescents & Adults with CHD Increased Mid Term Survival Lower Perioperative Mortality Advances in NICU Care Early Complete Repair Increased Early Survival Incidence of CHD

6 1970s: Introduction of echocardiography in clinical practice

7 Echo in ACHD: Role Establish or confirm anatomic diagnosis Assess new or residual lesions Assess valvular, atrial and ventricular function Guide transcatheter interventions Monitor intra & postoperative status Risk stratify for pregnancy

8 Echo in ACHD: Hints Understanding of CHD Original diagnosis and surgical procedures Examine the patient ECG and CXR Know & accept limitations of Echocardiography Combine with other imaging modalities

9 General Approach Start with standard views to get a lay of the land Follow blood flow through the heart as best possible Use clues, like pacemaker leads Evaluate residual stenoses, regurgitation, septal defects

10 The segmental approach Atria & venous connections Atrioventricular junction Concordance vs discordance Double inlet ventricle Atrioventricular valves Distinct valves, common valve Ventricles Ventriculoarterial junction Concordance vs discordance Double outlet Semilunar valves Great arteries

11 Specific Lesions Shunt lesions Obstructive lesions Complex CHD

12 Shunt lesions

13 ASDs The 2nd most common congenital heart disease in adults (incidence of 1 in 1,500 live births). ASD types include: Secundum ASD (80%) Primum ASD (15%) Sinus venosus defect SVC (5%) Sinus venosus defect IVC (1%) Unroofed coronary sinus (1%)

14 26 yo, male Asymptomatic Auscultation: mid-systolic murmur, Referred for echo (pre-participation athletic screening)

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17 Male, 60 yo NYHA II, palpitations since 9 months Known hole in the heart since 30 years ago ECG: SR, incomplete RBBB, right axis deviation

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21 Qp:Qs 2

22 Echo in transcatheter closure of ASD Male, 78 yo Permanent AF known since 2010 NYHA III Lower limb swelling so2 94% at rest NT-proBNP 1081 pg/ml TTE: Secundum ASD, RV volume overload, RVSP: 87 mmhg PVR: 2W, PVRi 3,15W/m2, Qp:Qs 3,6

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27 56 yo, male NYHA III Leg swelling, ascites

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29 RVSP 80 mmhg

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31 VSDs The most common congenital malformation (30% of all) Isolated or associated with other conditions VSD types: Perimembranous 80% Muscular 15-20% Subarterial 5%

32 36 yo, female, Asymptomatic, Loud holosystolic murmur left parasternal border

33 Perimembranous VSD

34 Perimembranous VSD

35 VSD Eisenmenger 30 yo, male, NYHA III, Cyanosis, clubbing so₂ 70%, Ht 62,3%

36 Muscular VSD

37 Muscular VSD

38 Repaired VSD

39 AVSDs Prevalence 2,9-7,4% 30% in Down syndrome Common ovoid shaped AV junction Five leaflet common AV valve Types Complete AVSD Partial (primum AVSDs): Atrial shunting only Fused superior and inferior leaflet attached to the crest of the VS

40 Complete AVSD 32 yo, female, Down syndrome, NYHA III, Cyanosis, so₂ 70 % at rest, Ht 59%

41 Complete AVSD

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44 2,4% of CHDs PDA Vessel distal to the left subclavian artery Shunting from the aorta to the pulmonary circulation Small PDAs generally asymptomatic Large PDAs LA, LV overload PH Eisenmenger syndrome

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46 PDA Eisenmenger 20 year-old, male Refugee from Iraq Suspected PH Shortness of breath on mild exertion (last 9 months)- WHO III Free past Medical History

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50 Obstructive lesions

51 LVOT obstruction BAV prevalence 1-2% in the population Subaortic Stenosis Supravalvular aortic stenosis

52 58 yo, female NYHA III Loud systolic murmur ECG: LV strain

53 Continuity equation

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56 Aortic coarctation Narrowing of the aorta distal to the left subclavian artery 5-8% of all congenital heart defects Associated with other lesions of the aorta (BAV), Turner and William syndrome, congenital rubella, Takayasu disease, neurofibromatosis

57 55 yo, male Resistant hypertension under 4 medications BP gradient between upper and lower extremities (>20 mmhg), Radiofemoral pulse delay Systolic murmur over the thoracic spine ECG: LV strain

58 RVOT obstruction

59 RVOT stenosis Supravalvular Commonly associated with ToF, Noonan syndrome, Williams-Beuren syndrome Valvular (80-90%) 9% of all CHD Subvalvular Associated with other conditions (ToF, Double chambered RV)

60 41 yo, male Valvuloplasty due to PS (1996) Systolic murmur

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62 Complex CHD

63 Tetralogy of Fallot (ToF) Most common cyanotic CHD 6,8% of all CHD Can be associated with: ASD (PoF) Right aortic arch Anomalous coronary arteries Di George syndrome

64 Uncorrected ToF Female, 17 yo Known heart murmur and a hole in the heart Multiple hospitalizations due to respiratory tract infections NYHA III Cyanosis, clubbing so₂ 83%

65 Uncorrected ToF

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68 Repaired ToF Echocardiographic assessment: PR severity Diastolic flow reversal in the branch pulmonary arteries, jet width, PRindex, Residual PS stenosis Severe: PG 64 mmhg, TRV > 3,5 m/sec RV function and dilation Residual VSD

69 Repaired ToF 45 yo, male NYHA II Ankle swelling ToF repair at the age of 10

70 ToF repaired

71 ToF repaired

72 d-tga Venticuloarterial discordance Atrioventricular concordance

73 d-tga Normal Circulation PA Ao PA dtga Ao PA Ao PV SVC/IVC PA SVC/IVC

74 Surgical repair Arterial switch Atrial switch Mustard operation Senning operation Rastelli Procedure

75 TGA- Arterial switch LV the systemic ventricle Operation: Transection of the great vessels above the valve sinus Switch of the arteries Translocation of the bifurcation of the PA anteriorly to the Aorta Re-implantation of the coronary arteries Echocardiographic assessment AV reg presence and severity PA narrowing at the anastomotic site or in the branches

76 Patient after arterial switch 17 yo, male NYHA II since 9 months, Diastolic heart murmur Arterial switch operation and VSD closure (2 months of age)

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80 TGA- Atrial switch RV the systemic ventricle Mustard/Senning operation: Redirect the venous blood on atrial level Systemic venous blood LV Pulmonary venous blood RV

81 TGA- Atrial switch Echocardiographic assessment: Narrowing or obstruction of the systemic venous channels decision for stenting in symptomatic pts Baffle leaks RV size and function TV regurgitation decision for surgery in symptomatic pts Parallel position of the great arteries

82 Patient after atrial switch 45yo, male Mustard operation (3 years old) (1976) NYHA II so₂ 94%

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85 cctga 1% of CHDs Double discordance: Atrioventricular & Ventriculoarterial Great arteries run parallel RV supports the systemic circulation 90% of pts have associated cardiac defects VSD Pulmonic stenosis Ebstein abnormality

86 26 yo, female Asymptomatic Routine control ECGs: P wave alternans

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89 31 yo, female cctga TV replacement + VSD closure + PPM implantation (2001)

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91 Ebstein s anomaly Rotation and apical displacement of the tricuspid orifice into the RV Atrialized RV, small functional RV Abnormal leaflets Echocardiographic assessment: Atrialized RV size TR severity (Systolic flow reversal in SVC and IVC) Decision for repair RVOTO by the anterior leaflet Atrial shunt lesions

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94 Univentricular heart One dominant functional pumping chamber Wide spectrum of conditions Double inlet LV (most common) Double inlet RV Tricuspid atresia Hypoplastic left & right heart syndrome Multiple associated malformations ASD, VSD, PDA LVOTO, RVOTO, Aortic coarctation Discordant ventriculoarterial connections

95 Fontan type operations Divert the systemic venous return to the PA without the interposition of a subpulmonary ventricle Echocardiographic assessment Dominant ventricle function and morphology AV valve regurgitation degree Arterial valves regurgitation or stenosis degree Patency of pulmonary pathways, Size of fenestrations

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98 Conclusion Adult congenital heart diseases are increasingly common Echocardiography is a mainstay of evaluation Knowing what to expect is optimal Referral to an ACHD center is recommended

99 Thank You