VSD

VSD classification according to it’s size:¹

1. Small VSD: <5 mm
2. Moderate VSD: 5–10 mm
3. Large VSD: >10 mm

Types of VSD:²

• Perimembranous (80%)
• Muscular (5–10%)
• Supracristal (5–8%)
• Posterior (8–10%)

*¹Step on to Paediatrics, Md Abid Hossain Mollah, 3rd Edition Page: 120;  ²Short and Long Cases in Clinical Medicine, HN Sarker Page: 58

As follows:

• Congenital (the most common)
  • VSD is the most common congenital heart disease
• Acquired
  • Rupture of interventricular septum after acute MI, rarely trauma.

* Long Cases in Clinical Medicine, ABM Abdullah Page: 242

Common site is the perimembranous part of intraventricular septum (in 90% cases).

* Long Cases in Clinical Medicine, ABM Abdullah Page: 242

As follows

• ASD
• VSD
• PDA
• Pulmonary edema

* Short and Long Cases in Clinical Medicine, HN Sarker Page: 58

Clinical manifestation

Small defect

• Patients usually remain asymptomatic with normal growth and development
• Incidental detection of a pansystolic murmur at left 3rd and 4th intercostal spaces

Large defect

• Dyspnoea at rest or on exertion
• Poor feeding/interrupted feeding
• Poor weight gain (failure to thrive)
• Easy fatigability
• Profuse perspiration (diaphoresis) e.g. head sweating
• Recurrent respiratory tract infections
• Cyanosls is usually absent

* Step on to Paediatrics, Md Abid Hossain Mollah, 3rd Edition Page: 121

VSD may be of three types according to the size. These are:

1. Small VSD (maladie de Roger): It is asymptomatic and usually closes spontaneously. But, there is a future risk of development of aortic regurgitation or endocarditis even after spontaneous closure. The systolic murmur is loud and prolonged.
2. Moderate VSD: The patient presents with fatigue and dyspnea. Heart is usually enlarged with a prominent apex beat. There is often a palpable systolic thrill and a loud “tearing” pansystolic murmur at the left lower sternal edge.
3. Large VSD: The murmur is soft. It may lead to pulmonary hypertension and Eisenmenger’s complex may result.

Note:

VSD may be associated with Turner’s syndrome, Down’s syndrome or maternal rubella during pregnancy.

* Long Cases in Clinical Medicine, ABM Abdullah Page: 242, 243

Patient may present as:

1. Cardiac failure in infants with congenital septal defect.
2. As a murmur with only minor hemodynamic disturbance in older children or adults.
3. Rarely as Eisenmenger’s syndrome.

* Pre-exam preparation for medicine, HN Sarker; Davidson’s Principles and Practice of Medicine, 22nd Edition Page: 634

General physical examination

• Appearance : Sick looking, often malnourished
• Respiratory rate : Increased
• Pulse : Rate: Increased, Volume: Good.
• Blood pressure: Normal
• Jugular venous pressure: May be raised
• Pedal oedema : Absent but may be present in heart failure

Precordium

• Inspection
  • Hyperdynamic, may be bulged
• Palpation
  • Apex beat is shifted to left (due to cardiomegaly) and is thrusting
  • Left parasternal heave may be present
  • Systolic thrill may be present in tricuspid area (related to grading of murmur)
  • P2 (pulmonary component of 2nd heart sound) may be palpable in pulmonary area
• Auscultation
  • 1st and 2nd heart sounds are audible in all 4 areas
  • A harsh pansystolic murmur (grade 4/6) best heard at lower left sternal border at the 3rd, 4th & 5th intercostal spaces. The murmur may radiate to the right lower sternal border. Intensity varies based on the size of the VSD and pulmonary vascular resistance

* Step on to Paediatrics, Md Abid Hossain Mollah, 3rd Edition Page: 121

Physical signs and auscultatory findings in VSD are:

• Heaving apex beat
• Left parasternal systolic thrill
• Pansystolic murmur in left sternal edge radiating over whole precordium.

* Pre-exam preparation for medicine, HN Sarker

No. Small VSD cause loud murmur (maladie de Roger) and large VSD is associated with soft murmur.

* Short and Long Cases in Clinical Medicine, HN Sarker Page: 58

As follows:

• X-ray chest
• ECG
• Echocardiography, preferably color doppler
• Cardiac catheterization may be necessary in some cases

* Short and Long Cases in Clinical Medicine, HN Sarker Page: 58

X-ray findings include

• Small defects
  • Normal heart size and pulmonary vascular markings (CRX may be normal)
• Larger defects
  • Cardiomegaly (cardiothoracic ratio >60%) with plethoric lung fields (increased pulmonary vascular markings with prominent main pulmonary arteries)
  • Cardiomegaly with oligemic lung fields (with development of pulmonary hypertension).

* Short and Long Cases in Clinical Medicine, HN Sarker Page: 58; Step on to Paediatrics, Md Abid Hossain Mollah, 3rd Edition Page: 121

Color Doppler echocardiogram. It shows location and size of the defect.

* Long Cases in Clinical Medicine, ABM Abdullah Page: 241

ECG

• Normal in small defect
• Left ventricular hypertrophy in large VSD
• Biventricular hypertrophy when associated with pulmonary hypertension
• P waves may be notched or peaked

* Step on to Paediatrics, Md Abid Hossain Mollah, 3rd Edition Page: 121, 122

Because of presence of systolic thrill and pansystolic murmur in the left lower parasternal area.

* Long Cases in Clinical Medicine, ABM Abdullah Page: 241

Yes, as apex beat is displaced and thrusting in character.

* Short and Long Cases in Clinical Medicine, HN Sarker Page: 58

Murmur is less loud than small VSD and presence of features of pulmonary hypertension.

* Short and Long Cases in Clinical Medicine, HN Sarker Page: 58

As follows:

• Mitral regurgitation (MR)
• Tricuspid regurgitation (TR).

* Long Cases in Clinical Medicine, ABM Abdullah Page: 241

In MR findings are:

• First heart sound is soft
• Pansystolic murmur in mitral area, which radiates towards the left axilla.

* Long Cases in Clinical Medicine, ABM Abdullah Page: 241

In TR, findings are:

• JVP may be raised. There may be giant V wave, oscillating up to the ear lobule.
• First heart sound is soft in tricuspid area.
• There is a pansystolic murmur in left lower parasternal area with no radiation and the murmur is louder with inspiration.
• Also there may be enlarged, tender, pulsatile liver.

Difference between VSD and TR:

* Long Cases in Clinical Medicine, ABM Abdullah Page: 241; Short and Long Cases in Clinical Medicine, HN Sarker Page: 58

Treatment

Counsel the parents about the disease, its complications and prognosis.

The goals of treatment are to–

• Ensure adequate growth of the patient
• Prevent the development of pulmonary vascular obstructive disease e. g. Eisenmenger syndrome
• To control congestive heart failure

Small defects

• Parents should be reassured of the relatively benign nature of the lesion
• Prophylaxis for infective endocarditis (penicillin).
• The child should be encouraged to live a normal life, with no restrictions on physical activity

Moderate to large defects (with pulmonary hypertension/ right ventricular failure/ left ventricular failure)

1. Supportive
   • Nutrition : High calorie diet (add fat and sugar) to ensure adequate weight gain. Occasionally, oral feeds must be supplemented with tube feeds
   • Diuretics: Frusemide (1–3 mg/kg/day divided in 2 or 3 doses) and/or Spironolactone may be used to relieve pulmonary congestion
   • Afterload reducing agents: ACE inhibitors
     • Enalapril (0.1–0.5 mg/kg/day – 12 hourly/once daily)
     • Captopril (0.05–0.1 mg/kg/dose – 8 hourly) may be used
   • Digoxin (5-10 μg/kg/day) may be indicated if diuresis and afterload reduction do not relieve symptoms of heart failure adequately
   • Treatment of pulmonary hypertension
2. Surgical
   • VSD closure (surgical or percutanesous) if no contraindications.

* Step on to Paediatrics, Md Abid Hossain Mollah, 3rd Edition Page: 122; Short and Long Cases in Clinical Medicine, HN Sarker Page: 59

Spontaneous closure of small defects occurs in approximately 35-50% patients, often in early childhood by 2 years of age (during the first year of life¹).

* Short and Long Cases in Clinical Medicine, HN Sarker Page: 58; ¹Step on to Paediatrics, Md Abid Hossain Mollah, 3rd Edition Page: 122

Irreversible severe pulmonary hypertension non responsive to pulmonary vasodilators, Eisenmenger’s syndrome (as it aggravates right sided heart failure).

Note:

Severe pulmonary hypertension is defined when pulmonary vascular resistance is 2/3 of systemic vascular resistance.

* Short and Long Cases in Clinical Medicine, HN Sarker Page: 59; Step on to Paediatrics, Md Abid Hossain Mollah, 3rd Edition Page: 122

If pulmonary hypertension is irreversible, closure is contraindicated.

* Short and Long Cases in Clinical Medicine, HN Sarker Page: 59

The indications include:

• Surgical closure at any age for large VSD where clinical symptoms and failure to thrive cannot be controlled medically
• Surgical closure between 6-12 months of age large VSD with pulmonary hypertension, even if symptoms controlled by medication
• Children older than 24 months with a Q_p:Q_s ratio > 2 : 1 (Pulmonary-Systemic flow ratio high i.e. Iarge left to right shunt)

Note:

In case of adult acute rupture o finterventricular septum, i.e. following myocardial infarction.

* Step on to Paediatrics, Md Abid Hossain Mollah, 3rd Edition Page: 122; Short and Long Cases in Clinical Medicine, HN Sarker Page: 59

The complications of VSD include

• Infective endocarditis
• Pulmonary hypertension
• Reversal of shunt (Eisenmenger’s syndrome)
• Left venuicular dysfunction/failure
• Aortic regurgitation (perimembranous or supracristal type)
• Ventricular arrhythmias.

Note:

When Eisenmenger’s syndrome develops, there is cyanosis, clubbing and evidence of pulmonary hypertension. Pansystolic murmur may disappear, because of equalization of pressure in right and left ventricle.

* Short and Long Cases in Clinical Medicine, HN Sarker Page: 58; Long Cases in Clinical Medicine, ABM Abdullah Page: 242