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Adult Congenital Heart Disease

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Essential Echocardiography
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Abstract

The number of patients with either uncorrected or repaired congenital cardiac lesions is on the rise, likely reflective of improvements in care and detection. With more patients surviving well into adulthood, they often will present for noncardiac surgery. Echocardiography remains central to lesion detection and aids in both surgical and device-based correction. It can also provide important prognostic information and quantify the effects (from the lesion) on ventricular function, pulmonary and systemic flow, etc. It is important for the beginner and intermediate practitioner to familiarize himself/herself with basic lesions encountered in adulthood, the echocardiographic techniques used to further identify and evaluate each condition, and the associated conditions that need to be sought and ruled out.

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Abbreviations

ASD:

Atrial septal defect

CFD:

Color flow Doppler

CHD:

Congenital heart disease

IAS:

Interatrial septum

IJ:

Internal jugular

LAX:

Long-axis

LVOT:

Left ventricular outflow tract

ME:

Midesophageal

PA:

Pulmonary artery

PASP:

Pulmonary artery systolic pressure

PDA:

Patent ductus arteriosus

PFO:

Patent foramen ovale

RV:

Right ventricle

RVOT:

Right ventricular outflow tract

SAX:

Short-axis

SVC:

Superior vena cava

TEE:

Transesophageal echocardiography

ToF:

Tetralogy of Fallot

TTE:

Transthoracic echocardiography

VSD:

Ventricular septal defect

References

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Correspondence to Swapnil Khoche .

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Electronic Supplementary Material

Midesophageal view of interatrial septum with color flow Doppler in a patient with a patent foramen ovale (PFO) (MP4 1229 kb)

Midesophageal view of interatrial septum during agitated saline injection demonstrating a patent foramen ovale (PFO) (MP4 5581 kb)

Midesophageal four-chamber view demonstrating an ostium secundum ASD with left-to-right flow demonstrated on color flow Doppler (MP4 1280 kb)

Midesophageal four-chamber view demonstrating an ostium primum ASD (MP4 1249 kb)

Midesophageal bicaval view in a patient with a superior sinus venosus ASD and a grossly dilated right atrium (RA). Color flow Doppler confirms left-to-right flow (MP4 228 kb)

Apical four-chamber view with a zoomed perspective of the interatrial septum with color compare feature (two-dimensional image on left; color flow Doppler applied on the right) in a patient with an ostium secundum ASD (MP4 1060 kb)

Midesophageal right ventricular inflow-outflow view in a patient with a perimembranous ventricular septal defect (VSD). Color flow Doppler confirms left-to-right flow (MP4 1015 kb)

Midesophageal long-axis view in a patient with an outlet VSD. Color flow Doppler demonstrates left to right flow from the left ventricular outflow tract to the right ventricular outflow tract (MP4 1536 kb)

Parasternal short-axis view at a basal level with color flow Doppler demonstrating a perimembranous VSD (MP4 425 kb)

Apical four-chamber view with an RV focus, zoomed perspective on interventricular septum with color compare feature (two-dimensional image on left; color flow Doppler applied on the right). Color flow Doppler demonstrates a muscular VSD (MP4 1169 kb)

Midesophageal two-chamber view with the dilated coronary sinus in cross section to the posterior aspect of the top of the left ventricle. Agitated saline injected into the left arm has resulted in “microbubbles” within the dilated coronary sinus, confirming a persistent left superior vena cava (MP4 3524 kb)

Midesophageal long-axis view in a patient with tetralogy of Fallot s/p VSD repair. Note the overriding aorta that remains and the evidence of right ventricular hypertrophy. Incidentally, there is a dilated coronary sinus in this patient that was a persistent left-sided superior vena cava (MP4 1091 kb)

Parasternal long-axis view in a patient with tetralogy of Fallot; note the presence of an overriding aorta. A perimembranous VSD is detected on color flow Doppler (MP4 558 kb)

Questions

Questions

  1. 1.

    Which of the following views provides the best visualization to detect shunt across a patent foramen ovale?

    1. A.

      Apical four-chamber view

    2. B.

      Transgastric midpapillary short-axis view

    3. C.

      Midesophageal bicaval view

    4. D.

      Midesophageal AV long-axis view

  2. 2.

    Which of the following is the most common kind of ventricular septal defect?

    1. A.

      Perimembranous

    2. B.

      Inlet

    3. C.

      Outlet

    4. D.

      Muscular

  3. 3.

    Which of the following congenital conditions in adults are amenable to device closure?

    1. A.

      Sinus venosus ASD

    2. B.

      Patent ductus arteriosus

    3. C.

      Complete AV cushion defect

    4. D.

      Situs inversus

  4. 4.

    Which of the following is a reason to use a Valsalva maneuver during a bubble study to detect a patent foramen ovale?

    1. A.

      To increase the turbulence of the blood across a shunt

    2. B.

      To induce a right-to-left shunt

    3. C.

      To enable positioning of the echo probe closer to the interatrial septum

    4. D.

      To reduce transit time of the echo contrast to reach the atrium

  5. 5.

    A 25-year-old woman is diagnosed with a ventricular septal defect, moderate tricuspid regurgitation, and no other valvular abnormalities. The peak velocity across the VSD is measured at 4 m/s. Her right atrial pressure (or CVP) is 4 mmHg, and her systolic BP is 110/70 mmHg. What is the peak systolic pulmonary pressure?

    1. A.

      46 mmHg

    2. B.

      50 mmHg

    3. C.

      64 mmHg

    4. D.

      94 mmHg

  6. 6.

    Which of the following is most true regarding restrictive VSDs?

    1. A.

      Compared to a nonrestrictive defect, they are easier to detect using 2D imaging.

    2. B.

      Reduction in the Nyquist limit using color flow Doppler aids in detection.

    3. C.

      Restrictive VSDs are generally less severe than nonrestrictive defects.

    4. D.

      Restrictive VSDs exhibit non-turbulent flow.

  7. 7.

    In the midesophageal bicaval view, following release of a Valsalva maneuver, which of the following would be expected regarding flow through a patent foramen ovale?

    1. A.

      Flow should be toward the probe and the right side of the display.

    2. B.

      Flow does not occur due to closure of the flap between the septum secundum and septum primum.

    3. C.

      Flow is more difficult to detect with the color scale reduced to 30 cm/s.

    4. D.

      Flow can only be detected with bubble contrast.

  8. 8.

    Which of the following conditions does not result in the dilation of the coronary sinus?

    1. A.

      Unroofed coronary sinus

    2. B.

      Persistent left SVC

    3. C.

      Severe right ventricular pressure or volume overload

    4. D.

      Presence of a Eustachian valve

  9. 9.

    Which of the following is most true regarding detection of VSDs with echocardiography?

    1. A.

      Transthoracic views are not sensitive for small VSDs.

    2. B.

      A low Qp/Qs indicates that there is a right-to-left shunt.

    3. C.

      Bubble studies are generally not used for detection due to the risk for air emboli.

    4. D.

      Flow is usually right to left.

  10. 10.

    Which of the following describes an ostium primum ASD?

    1. A.

      It is the most common type of ASD.

    2. B.

      It results from failure of fusion between the septum primum and the septum secundum.

    3. C.

      It is the result of incomplete fusion between the septum primum and the endocardial cushion.

    4. D.

      It is a communication between the ostium primum and the ostium secundum.

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Khoche, S. (2022). Adult Congenital Heart Disease. In: Maus, T.M., Tainter, C.R. (eds) Essential Echocardiography. Springer, Cham. https://doi.org/10.1007/978-3-030-84349-6_18

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  • DOI: https://doi.org/10.1007/978-3-030-84349-6_18

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  • Publisher Name: Springer, Cham

  • Print ISBN: 978-3-030-84348-9

  • Online ISBN: 978-3-030-84349-6

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