Kyphoscoliotic heart disease

Cardiovascular

Clinicals - History

Fact Explanation
Difficulty in breathing Hypoxemia due to hypoventilation in kyphoscoliosis leads to pulmonary vasoconstriction and pulmonary hypertension which give rise to difficulty in breathing Difficulty in breathing
Hypoxemia due to hypoventilation in kyphoscoliosis leads to pulmonary vasoconstriction and pulmonary hypertension which give rise to difficulty in breathing
Fatigue Reduced oxygen to tissues due to hypoxaemia Fatigue
Reduced oxygen to tissues due to hypoxaemia
Ankle swelling Kyphoscoliotic diseases leads to pulmonary hypertension,which increases the work of the right ventricle, which leads more or less rapidly to right ventricular
enlargement (associating hypertrophy and dilatation) which
can result in ventricular dysfunction (systolic, diastolic). Later,
right heart failure (RHF) ) characterised by the presence of peripheral edema
Ankle swelling
Kyphoscoliotic diseases leads to pulmonary hypertension,which increases the work of the right ventricle, which leads more or less rapidly to right ventricular
enlargement (associating hypertrophy and dilatation) which
can result in ventricular dysfunction (systolic, diastolic). Later,
right heart failure (RHF) ) characterised by the presence of peripheral edema
History of chronic obstructive pulmonary disease / interstitial lung disease These can lead to pulmonary hypertension due to changes in lung tissues History of chronic obstructive pulmonary disease / interstitial lung disease
These can lead to pulmonary hypertension due to changes in lung tissues
History of Ehlers-Danlos Syndrome Ehlers-Danlos syndrome (EDS), kyphoscoliotic form (previously known as EDS VI) is a generalized connective tissue disorder.and respiratory compromise if kyphoscoliosis is severe History of Ehlers-Danlos Syndrome
Ehlers-Danlos syndrome (EDS), kyphoscoliotic form (previously known as EDS VI) is a generalized connective tissue disorder.and respiratory compromise if kyphoscoliosis is severe
Family history Kyphoscoliosis can be seen in several inherited connective tissue disorders.eg: EDS, kyphoscoliotic form is inherited in an autosomal recessive manner Family history
Kyphoscoliosis can be seen in several inherited connective tissue disorders.eg: EDS, kyphoscoliotic form is inherited in an autosomal recessive manner

Clinicals - Examination

Fact Explanation
Dyspnoea Hypoxemia due to hypoventilation in kyphoscoliosis leads to pulmonary vasoconstriction and pulmonary hypertension which give rise to difficulty in breathing Dyspnoea
Hypoxemia due to hypoventilation in kyphoscoliosis leads to pulmonary vasoconstriction and pulmonary hypertension which give rise to difficulty in breathing
Spinal defects Connective tissues disorders
Kyphosis is excessive curvature of the spine in the sagittal (A-P) plane.
Scoliosis is abnormal curvature of the spine in the coronal (lateral) plane.
Lordosis or hyperlordosis is excessive curving of the lower spine and is often associated with scoliosis or kyphosis
Kyphoscoliosis A scoliosis accompanied by a true hyperkyphosis
Spinal defects
Connective tissues disorders
Kyphosis is excessive curvature of the spine in the sagittal (A-P) plane.
Scoliosis is abnormal curvature of the spine in the coronal (lateral) plane.
Lordosis or hyperlordosis is excessive curving of the lower spine and is often associated with scoliosis or kyphosis
Kyphoscoliosis A scoliosis accompanied by a true hyperkyphosis
Barrel shaped chest Due to increased anteroposterior diameter Barrel shaped chest
Due to increased anteroposterior diameter
Friable, hyperextensible skin, thin scars, easy bruising Skin changes in Ehlers-Danlos Syndrome Friable, hyperextensible skin, thin scars, easy bruising
Skin changes in Ehlers-Danlos Syndrome
Ankle oedema , ascites Kyphoscoliotic diseases leads to pulmonary hypertension,which increases the work of the right ventricle, which leads more or less rapidly to right ventricular
enlargement (associating hypertrophy and dilatation) which
can result in ventricular dysfunction (systolic, diastolic). Later,
right heart failure (RHF) ) characterised by the presence of peripheral edema
Ankle oedema , ascites
Kyphoscoliotic diseases leads to pulmonary hypertension,which increases the work of the right ventricle, which leads more or less rapidly to right ventricular
enlargement (associating hypertrophy and dilatation) which
can result in ventricular dysfunction (systolic, diastolic). Later,
right heart failure (RHF) ) characterised by the presence of peripheral edema
Engorged neck veins Due to right heart failure Engorged neck veins
Due to right heart failure
A murmur of tricuspid regurgitation Pansystolic heart murmur.Best heard over lower left sternal border.Suggesting right ventricular dilatation, is a very late sign in respiratory patients A murmur of tricuspid regurgitation
Pansystolic heart murmur.Best heard over lower left sternal border.Suggesting right ventricular dilatation, is a very late sign in respiratory patients
Changes in heart sounds Due to pulmonary hypertension.Widely split S2 or second heart sound, a loud P2 or pulmonic valve closure sound Changes in heart sounds
Due to pulmonary hypertension.Widely split S2 or second heart sound, a loud P2 or pulmonic valve closure sound
Cyanosis Hypoxemia due to hypoventilation Cyanosis
Hypoxemia due to hypoventilation
Polycythemia Arterial oxygen desaturation
causes a relative and absolute increase in red
cell mass: erythrocytosis (secondary polycythemia)
Polycythemia
Arterial oxygen desaturation
causes a relative and absolute increase in red
cell mass: erythrocytosis (secondary polycythemia)

Investigations - Diagnosis

Fact Explanation
Electrocardiogram Right ventricular hypertrophy-
Right axis deviation (> 90 degrees) in presence of disease capable of causing RVH
R in aVR ≥ 5 mm, or
R in aVR > Q in aVR
Any one of the following in lead V1:
R/S ratio > 1 and negative T wave
qR pattern
R > 6 mm, or S < 2mm, or rSR' with R' > 10 mm
Electrocardiogram
Right ventricular hypertrophy-
Right axis deviation (> 90 degrees) in presence of disease capable of causing RVH
R in aVR ≥ 5 mm, or
R in aVR > Q in aVR
Any one of the following in lead V1:
R/S ratio > 1 and negative T wave
qR pattern
R > 6 mm, or S < 2mm, or rSR' with R' > 10 mm
Chest x ray Right atrial and ventricular enlargement Chest x ray
Right atrial and ventricular enlargement
Echocardiogram Wall thickness,chamber dilatations,ejection fraction can be measured.Valve insufficiencies can be identified Echocardiogram
Wall thickness,chamber dilatations,ejection fraction can be measured.Valve insufficiencies can be identified

Investigations - Management

Fact Explanation
Electrocardiogram Right ventricular hypertrophy-
Right axis deviation (> 90 degrees) in presence of disease capable of causing RVH
R in aVR ≥ 5 mm, or
R in aVR > Q in aVR
Any one of the following in lead V1:
R/S ratio > 1 and negative T wave
qR pattern
R > 6 mm, or S < 2mm, or rSR' with R' > 10 mm
Electrocardiogram
Right ventricular hypertrophy-
Right axis deviation (> 90 degrees) in presence of disease capable of causing RVH
R in aVR ≥ 5 mm, or
R in aVR > Q in aVR
Any one of the following in lead V1:
R/S ratio > 1 and negative T wave
qR pattern
R > 6 mm, or S < 2mm, or rSR' with R' > 10 mm
Echocardiogram Wall thickness,chamber dilatations,ejection fraction can be measured.Valve insufficiencies can be identified Echocardiogram
Wall thickness,chamber dilatations,ejection fraction can be measured.Valve insufficiencies can be identified
Full blood count To assess general condition of the patient and hemoglobin levels. Full blood count
To assess general condition of the patient and hemoglobin levels.
Chest X ray Vertebral defects can be visualised.
Also right atrial and ventricular enlargements can be visualised
Chest X ray
Vertebral defects can be visualised.
Also right atrial and ventricular enlargements can be visualised
Spirometry Forced Expiratory Volume in One Second (FEV1)/Forced Vital Capacity (FVC) < 0.7
FEV1 >/= 80% predicted
Spirometry
Forced Expiratory Volume in One Second (FEV1)/Forced Vital Capacity (FVC) < 0.7
FEV1 >/= 80% predicted

Management - Supportive

Fact Explanation
Life style modifications Stop smoking as smoking further worsen the lung diseases Life style modifications
Stop smoking as smoking further worsen the lung diseases
Control of other co morbid conditions Controlling of diabetes,dyslipidemia etc. as these conditions can aggravate the heart diseases Control of other co morbid conditions
Controlling of diabetes,dyslipidemia etc. as these conditions can aggravate the heart diseases
Treating respiratory failure Patients with kyphoscoliosis and chronic respiratory insufficiency are treated either with home oxygen therapy or ventilation.
nocturnal nasal intermittent positive pressure ventilation, plus long term oxygen therapy results in more favourable survival and changes in blood gases and respiratory function than long-term oxygen therapy alone
Treating respiratory failure
Patients with kyphoscoliosis and chronic respiratory insufficiency are treated either with home oxygen therapy or ventilation.
nocturnal nasal intermittent positive pressure ventilation, plus long term oxygen therapy results in more favourable survival and changes in blood gases and respiratory function than long-term oxygen therapy alone

Management - Specific

Fact Explanation
β-receptor antagonists, inotropes, and diuretics To treat heart failure by reducing afterload β-receptor antagonists, inotropes, and diuretics
To treat heart failure by reducing afterload
Treatment of the lung disease Oxygen (controlled oxygen),
Pulmonary vasodilators (Sildenafil),
Inhaled nitric oxide- is a more potent vasodilator than oxygen,
Reduction in hematocrit - Phlebotomy is indicated in patients with a severe elevation in hematocrit not responding to long term oxygen therapy
Treatment of the lung disease
Oxygen (controlled oxygen),
Pulmonary vasodilators (Sildenafil),
Inhaled nitric oxide- is a more potent vasodilator than oxygen,
Reduction in hematocrit - Phlebotomy is indicated in patients with a severe elevation in hematocrit not responding to long term oxygen therapy
Correction of kyphoscoliosis Back braces Correction of kyphoscoliosis
Back braces
Surgical methods To correct kyphoscoliosis
Vertebral column resection for the treatment of severe spinal deformity
Vertebral column decancellation for the management of sharp angular spinal deformity
Staged corrective surgery for complex adolescent kyphoscoliosis due to contractures
Thoracic pedicle screw fixation
Surgical methods
To correct kyphoscoliosis
Vertebral column resection for the treatment of severe spinal deformity
Vertebral column decancellation for the management of sharp angular spinal deformity
Staged corrective surgery for complex adolescent kyphoscoliosis due to contractures
Thoracic pedicle screw fixation

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