What are the health implications of Harrison's sulcus with pectus carinatum?

Harrison's Sulcus with Pectus Carinatum: Health Implications

Harrison's sulcus combined with pectus carinatum indicates underlying chest wall structural abnormalities that can cause restrictive respiratory impairment, require evaluation for associated genetic syndromes (particularly Marfan and Noonan syndromes), and may necessitate cardiac assessment due to potential congenital heart defects or mitral valve prolapse. 1, 2

Primary Respiratory Consequences

The combination of Harrison's sulcus with pectus carinatum creates thoracic restriction that impairs pulmonary mechanics, with the restrictive pattern primarily affecting the lateral thorax rather than being solely related to spinal deformities. 1

• Chest wall deformities including rib crowding, pectus carinatum, and more horizontal rib positioning (from thoracic vertebral collapse) contribute to restrictive lung disease in up to 60% of affected patients 1
• The restriction is entirely located in the thorax at rest and during maximal maneuvers, with patients compensating by shifting respiratory expansion to the abdomen 1
• In patients with osteogenesis imperfecta who have these deformities, the restrictive respiratory pattern may be more severe, with ventilatory mechanics altered mainly in the lateral thorax 2, 3
• There is no direct correlation between the appearance of chest wall deformities and clinical symptoms, making objective pulmonary function testing essential 4

Mandatory Cardiac Evaluation

Complete cardiac evaluation is mandatory when Harrison's sulcus and pectus carinatum coexist, as this combination occurs in 0.17% of patients with congenital heart disease and may indicate underlying genetic syndromes. 2, 5

• Associated congenital heart defects occur in documented cases, with only 19 cases reported historically but representing a significant clinical concern 5
• Mitral valve prolapse can be associated with pectus excavatum/carinatum, especially in connective tissue syndromes 3
• In Noonan syndrome patients, pectus carinatum occurs alongside cardiac alterations such as pulmonary stenosis 2, 3
• Kyphoscoliosis combined with pectus deformities may cause cardiac migration to the left hemithorax, which affects both diagnosis and surgical planning 2, 3

Genetic Syndrome Screening

Screen for Marfan syndrome and Noonan syndrome as these are the most important and frequently observed monogenic conditions associated with pectus carinatum. 6

Marfan Syndrome Indicators:

• Pectus carinatum scores 2 points in the systemic features scoring system (pectus excavatum scores 1 point) 1
• Look for ectopia lentis, aortic root dilation, wrist and thumb signs, hindfoot deformity, dural ectasia, and protrusio acetabuli 1
• These patients require thorough aortic assessment with annual echocardiography and consideration for β-blocker therapy 1, 2
• Aortic root dilation is usually mild but requires long-term monitoring 1

Noonan Syndrome Indicators:

• Dysplastic pulmonary valve with marked myxomatous thickening may be present 1
• Comprehensive cardiac evaluation is essential due to high prevalence of pulmonary stenosis 2

Surgical Considerations and Complications

If surgical correction is planned, timing relative to any cardiac surgery is critical—repair pectus excavatum first if extracardiac conduit placement is anticipated, otherwise repair chest wall deformity after cardiac surgery. 5

• Avoid simultaneous cardiac and pectus repair due to potential major complications including chest wall instability and intrathoracic hemorrhage 5
• The sandwich technique for combined pectus carinatum with Harrison sulcus shows mean surgical duration of 179 minutes, minimal blood loss (10 mL), and 7.89% complication rate 7
• Pleural or pericardial entry complications and atelectasis are potential postoperative concerns 3, 5
• For patients requiring extracardiac conduits, initial pectus repair followed by cardiac repair at 6+ weeks eliminates possible extrinsic compression of the conduit by depressed sternum 5

Evaluation Algorithm

Perform detailed dysmorphological physical examination looking specifically for:

• Joint hypermobility, skin hyperextensibility, and arachnodactyly (Marfan features) 1
• Dolichocephaly, rachitic rosary, and limb deformities (rickets/metabolic bone disease) 1
• Scoliosis (present in 26% of pectus carinatum cases) and other musculoskeletal abnormalities 8
• Family history of chest wall deformities (positive in 26% of cases) 8

Obtain baseline pulmonary function testing when the child can cooperate (typically age 5+), looking for FVC reduction and FEV1/FVC ratio abnormalities. 1

Order echocardiography to assess for aortic root dilation, mitral valve prolapse, and congenital heart defects, with MRI or CT for surgical planning if intervention is considered. 2, 3